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Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:43:58 UTC
Secondary Accession Numbers
  • HMDB00225
Metabolite Identification
Common NameOxoadipic acid
Description2-Oxoadipic acid is produced from lysine in the cytosol of cells via the saccharopine and the pipecolic acid pathways. Catabolites of hydroxylysine and tryptophan enter these pathways as 2-aminoadipic- -semialdehyde and 2-oxoadipate, respectively. In the matrix of mitochondria, 2-oxoadipate is decarboxylated to glutaryl-CoA by the 2-oxoadipate dehydrogenase complex and then converted to acetyl-CoA. 2-Oxoadipic aciduria is an in-born error of metabolism of lysine, tryptophan, and hydroxylysine, in which abnormal quantities of 2-aminoadipic acid are found in body fluids along with 2-oxoadipic acid. Patients with 2-Oxoadipic acidemias are mentally retarded with hypotonia or seizures. 2-Oxoadipic aciduria can occur in patients with Kearns-Sayre Syndrome, a progressive disorder with onset prior to 20 years of age in which multiple organ systems are affected, including progressive external ophthalmoplegia, retinopathy, and the age of onset, and these are associated classically with abnormalities in cardiac conduction, cerebellar signs, and elevated cerebrospinal fluid protein (PMID: 10655159 , 16183823 , 11083877 ). Oxoadipic acid is found to be associated with alpha-aminoadipic aciduria, which is an inborn error of metabolism.
2-Oxoadipic acidKegg
2-Ketoadipic acidHMDB
2-oxo-Hexanedioic acidHMDB
2-Oxohexanedioic acidHMDB
2-Oxohexanedionic acidHMDB
a-Ketoadipic acidHMDB
a-Oxoadipic acidHMDB
alpha-Ketoadipic acidHMDB
alpha-Oxoadipic acidHMDB
Chemical FormulaC6H8O5
Average Molecular Weight160.1247
Monoisotopic Molecular Weight160.037173366
IUPAC Name2-oxohexanedioic acid
Traditional Nameoxoadipate
CAS Registry Number3184-35-8
InChI Identifier
Chemical Taxonomy
Description belongs to the class of organic compounds known as medium-chain keto acids and derivatives. These are keto acids with a 6 to 12 carbon atoms long side chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassMedium-chain keto acids and derivatives
Direct ParentMedium-chain keto acids and derivatives
Alternative Parents
  • Medium-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
Physiological effect

Health effect:


Route of exposure:


Biological location:


Indirect biological role:

Physical Properties
Experimental Properties
Melting Point127 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility23.4 g/LALOGPS
pKa (Strongest Acidic)2.84ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area91.67 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity33.48 m³·mol⁻¹ChemAxon
Polarizability14.16 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-002b-3910000000-66f39a8b247786a425e6JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-002k-3910000000-977efef811bb47535b80JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udr-6900000000-0eb27647b831277a2aefJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udr-6900000000-46cd4d782e3d19030993JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-002b-3910000000-66f39a8b247786a425e6JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-002k-3910000000-977efef811bb47535b80JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udr-6900000000-0eb27647b831277a2aefJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udr-6900000000-46cd4d782e3d19030993JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00r6-9300000000-b3f156c173adabf3f1bbJSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-0079-9830000000-a3b1d5e6d378582a6fc2JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4i-0900000000-aef94cc19e461712a097JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0bt9-9500000000-9326906313591bb9da1cJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4i-9000000000-05db676896bb1e350497JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4l-9000000000-1e566f572b14692461f2JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-527da1c1c271db4d8db4JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-0a4i-9600000000-38ade0e080ecb7a89588JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0006-2900000000-757eb57780db9df4f78dJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-016v-9700000000-675e5732632f0a0cabe1JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-1da8154067fdb9a27459JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a4i-1900000000-0e26a52af95eb287809dJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-066v-7900000000-d898e0593464c13b3f89JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-052g-9100000000-34782bad1c8755ac897dJSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR13C NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen Locations
  • Blood
  • Urine
Tissue LocationsNot Available
Normal Concentrations
BloodExpected but not Quantified Not AvailableNot Available
    UrineDetected and Quantified1.2 umol/mmol creatinineChildren (1-13 years old)Both
    UrineDetected and Quantified0.17 (0.02-0.57) umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified0.21 (0.04-0.81) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified1.3 (1.0-1.5) umol/mmol creatinineChildren (1-13 years old)Both
    Abnormal Concentrations
    UrineDetected and Quantified520-970 umol/mmol creatinineChildren (1-13 years old)Female
    2-Aminoadipic aciduria
    UrineDetected and Quantified62 umol/mmol creatinineAdolescent (13-18 years old)Malealpha-Ketoadipic Aciduria details
    UrineDetected and Quantified20-120 umol/mmol creatinineChildren (1-13 years old)Not Specifiedalpha-Aminoadipic aciduria details
    UrineDetected and Quantified456 umol/mmol creatinineInfant (0-1 year old)Not Specified
    alpha-ketoadipic aciduria
    UrineDetected and Quantified135 umol/mmol creatinineNewborn (0-30 days old)Not Specified
    alpha-ketoadipic aciduria
    Associated Disorders and Diseases
    Disease References
    2-Ketoadipic acidemia
    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 ]
    2. Peng H, Shinka T, Inoue Y, Mitsubuchi H, Ishimatsu J, Yoshino M, Kuhara T: Asymptomatic alpha-ketoadipic aciduria detected during a pilot study of neonatal urine screening. Acta Paediatr. 1999 Aug;88(8):911-4. [PubMed:10503694 ]
    3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: METAGENE consortium.
    Alpha-aminoadipic aciduria
    1. Gray RG, O'Neill EM, Pollitt RJ: Alpha-aminoadipic aciduria: chemical and enzymatic studies. J Inherit Metab Dis. 1980;2(4):89-92. [PubMed:6796766 ]
    2-Aminoadipic aciduria
    1. Danhauser K, Sauer SW, Haack TB, Wieland T, Staufner C, Graf E, Zschocke J, Strom TM, Traub T, Okun JG, Meitinger T, Hoffmann GF, Prokisch H, Kolker S: DHTKD1 mutations cause 2-aminoadipic and 2-oxoadipic aciduria. Am J Hum Genet. 2012 Dec 7;91(6):1082-7. doi: 10.1016/j.ajhg.2012.10.006. Epub 2012 Nov 8. [PubMed:23141293 ]
    Associated OMIM IDs
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB003362
    KNApSAcK IDC00000770
    Chemspider ID70
    KEGG Compound IDC00322
    BioCyc IDNot Available
    BiGG ID1485244
    Wikipedia LinkNot Available
    METLIN ID5234
    PubChem Compound71
    PDB IDNot Available
    ChEBI ID15753
    Food Biomarker OntologyNot Available
    VMH IDNot Available
    Synthesis ReferenceNelson, Randall B.; Gribble, Gordon W. Preparation of a-ketoadipic acid. Organic Preparations and Procedures International (1973), 5(2), 55-8.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. 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 ]
    2. 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 ]
    3. Barshop BA, Nyhan WL, Naviaux RK, McGowan KA, Friedlander M, Haas RH: Kearns-Sayre syndrome presenting as 2-oxoadipic aciduria. Mol Genet Metab. 2000 Jan;69(1):64-8. [PubMed:10655159 ]
    4. Schulze-Bergkamen A, Okun JG, Spiekerkotter U, Lindner M, Haas D, Kohlmuller D, Mayatepek E, Schulze-Bergkamen H, Greenberg CR, Zschocke J, Hoffmann GF, Kolker S: Quantitative acylcarnitine profiling in peripheral blood mononuclear cells using in vitro loading with palmitic and 2-oxoadipic acids: biochemical confirmation of fatty acid oxidation and organic acid disorders. Pediatr Res. 2005 Nov;58(5):873-80. Epub 2005 Sep 23. [PubMed:16183823 ]
    5. Fiermonte G, Dolce V, Palmieri L, Ventura M, Runswick MJ, Palmieri F, Walker JE: Identification of the human mitochondrial oxodicarboxylate carrier. Bacterial expression, reconstitution, functional characterization, tissue distribution, and chromosomal location. J Biol Chem. 2001 Mar 16;276(11):8225-30. Epub 2000 Nov 16. [PubMed:11083877 ]


    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:
    Uniprot ID:
    Molecular weight:
    General function:
    Involved in transferase activity, transferring nitrogenous groups
    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).
    Gene Name:
    Uniprot ID:
    Molecular weight:
    Aminoadipic acid + Oxoglutaric acid → Oxoadipic acid + L-Glutamic aciddetails
    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:
    Uniprot ID:
    Molecular weight:
    Oxoadipic acid + Coenzyme A + NAD → Glutaryl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
    Oxoadipic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine + Carbon dioxidedetails
    General function:
    Specific function:
    Not Available
    Gene Name:
    Uniprot ID:
    Molecular weight:
    General function:
    Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
    Specific function:
    Not Available
    Gene Name:
    Uniprot ID:
    Molecular weight:
    Not Available
    Oxoadipic acid + Coenzyme A + NAD → Glutaryl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
    Oxoadipic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine + Carbon dioxidedetails