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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:56 UTC
HMDB IDHMDB0000208
Secondary Accession Numbers
Metabolite Identification
Common NameOxoglutaric acid
Description
Structure
Thumb
Synonyms
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
Physiological effect
Disposition
Biological locationRoute of exposureSource
Process
Naturally occurring process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point115.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility541.5 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg121.030932474
[M-H]-Not Available122.4http://allccs.zhulab.cn/database/detail?ID=AllCCS00001766
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
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 ]
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 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
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.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
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 ]
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 ]
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 ]
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)
  • 114500 (Colorectal cancer)
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 610247 (Eosinophilic esophagitis)
  • 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 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
Food Biomarker OntologyNot Available
VMH IDAKG
MarkerDB IDMDB00000099
Good Scents IDNot Available
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

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

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Gene Name:
DLD
Uniprot ID:
P09622
Molecular weight:
54176.91
General function:
Involved in 4-aminobutyrate transaminase activity
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.
Gene Name:
ABAT
Uniprot ID:
P80404
Molecular weight:
56438.405
Reactions
gamma-Aminobutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + Glutamic aciddetails
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid → 2-Methyl-3-oxopropanoic acid + Glutamic aciddetails
beta-Alanine + Oxoglutaric acid → Malonic semialdehyde + Glutamic aciddetails
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + Glutamic aciddetails
General function:
Involved in electron carrier activity
Specific function:
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:
PHYH
Uniprot ID:
O14832
Molecular weight:
38538.065
Reactions
Phytanoyl-CoA + Oxoglutaric acid + Oxygen → 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2)details
General function:
Involved in 1-aminocyclopropane-1-carboxylate synthase activity
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 towards phenylalanine.
Gene Name:
TAT
Uniprot ID:
P17735
Molecular weight:
50398.895
Reactions
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + Glutamic aciddetails
Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + Glutamic aciddetails
2-Oxo-4-methylthiobutanoic acid + Glutamic acid → Methionine + Oxoglutaric aciddetails
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 + Glutamic aciddetails
Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + Glutamic aciddetails
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + Glutamic aciddetails
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + Glutamic aciddetails
3-Sulfinoalanine + Oxoglutaric acid → 3-Sulfinylpyruvic acid + Glutamic aciddetails
4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + Glutamic aciddetails
General function:
Involved in transferase activity, transferring nitrogenous groups
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.
Gene Name:
GOT2
Uniprot ID:
P00505
Molecular weight:
47517.285
Reactions
L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + Glutamic aciddetails
Kynurenine + Oxoglutaric acid → 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + Glutamic aciddetails
Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + Glutamic aciddetails
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + Glutamic aciddetails
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + Glutamic aciddetails
3-Sulfinoalanine + Oxoglutaric acid → 3-Sulfinylpyruvic acid + Glutamic aciddetails
4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + Glutamic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
ALDH2
Uniprot ID:
P05091
Molecular weight:
56380.93
Reactions
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid.
Gene Name:
ALDH3A2
Uniprot ID:
P51648
Molecular weight:
54847.36
Reactions
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation.
Gene Name:
ALDH1B1
Uniprot ID:
P30837
Molecular weight:
57248.96
Reactions
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Reactions
Leucine + Oxoglutaric acid → Ketoleucine + Glutamic aciddetails
Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic aciddetails
L-Valine + Oxoglutaric acid → alpha-Ketoisovaleric acid + Glutamic aciddetails

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