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Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-26 21:22:09 UTC
Secondary Accession Numbers
  • HMDB00408
Metabolite Identification
Common Name2-Methyl-3-ketovaleric acid
Description2-Methyl-3-ketovaleric acid is a metabolite of beta-leucine in the beta-keto pathway of leucine metabolism. 2-Methyl-3-ketovaleric acid is a known pathological metabolite and associated with propionic acidemia, especially during periods of ketoacidosis; a severe deficiency of propionyl-CoA carboxylase in cultured fibroblasts has been detected in some neonatal cases. (PMID 7389125 , 6820422 , 7119896 , 7430116 , 3356699 ). 2-Methyl-3-ketovaleric acid induces a strong activity of inosine-5'-monophosphate dehydrogenase (IMPDH; the rate-limiting enzyme in GTP synthesis), mimicking glucose. (PMID: 11145582 ).
2-Methyl-3-oxo-pentanoic acidChEBI
2-Methyl-3-oxovaleric acidChEBI
beta-Ketoisocaproic acidChEBI
b-Ketoisocaproic acidGenerator
Β-ketoisocaproic acidGenerator
Chemical FormulaC6H10O3
Average Molecular Weight130.1418
Monoisotopic Molecular Weight130.062994186
IUPAC Name2-methyl-3-oxopentanoic acid
Traditional Name2-methyl-3-oxovaleric acid
CAS Registry Number14925-93-0
InChI Identifier
Chemical Taxonomy
Description belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassShort-chain keto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
  • Beta-keto acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Beta-hydroxy ketone
  • Fatty acyl
  • 1,3-dicarbonyl compound
  • Ketone
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors


Biological location:


Naturally occurring process:


Industrial application:

Biological role:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility30 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility60.6 g/LALOGPS
pKa (Strongest Acidic)4.32ChemAxon
pKa (Strongest Basic)-7.6ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity31.74 m³·mol⁻¹ChemAxon
Polarizability12.95 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0a4i-9100000000-7fc8c1cd090e8a94323bSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00g3-8900000000-6b17fb77410d2c11984cSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-2900000000-b53ef30491933435a749Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-4900000000-3c46dc3d99a57de6904eSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-001i-6900000000-ed7da1f432da0405f0b9Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01qi-4900000000-a3f116ad9911c34a5e5aSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01p9-9300000000-b6eb6753b3767b8c4354Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-056r-9000000000-daaf55f6c5e553cfd618Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-5900000000-429ffb363a166c7cfbb6Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-000i-9000000000-4e936ea24083981286feSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0aor-9000000000-fb4f1d84bfa6c035ededSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Urine
Tissue LocationsNot Available
Normal Concentrations
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified<2 umol/mmol creatinineChildren (1 - 18 years old)Both
    • BC Children's Hos...
UrineDetected and Quantified1.1(0.8-1.3) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothAutosomal dominant polycystic kidney disease (ADPKD) details
Associated Disorders and Diseases
Disease References
Autosomal dominant polycystic kidney disease
  1. Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Associated OMIM IDs
  • 601313 (Autosomal dominant polycystic kidney disease)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022024
KNApSAcK IDNot Available
Chemspider ID164251
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
PubChem Compound189028
PDB IDNot Available
ChEBI ID86365
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB ID
Synthesis ReferenceHirai, Yasuhiro; Aida, Takuzo; Inoue, Shohei. Artificial photosynthesis of b-ketocarboxylic acids from carbon dioxide and ketones via enolate complexes of aluminum porphyrin. , Journal of the American Chemical Society (1989), 111(8), 3062-3.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Kuhara T, Shinka T, Matsuo M, Matsumoto I: Increased excretion of lactate, glutarate, 3-hydroxyisovalerate and 3-methylglutaconate during clinical episodes of propionic acidemia. Clin Chim Acta. 1982 Aug 4;123(1-2):101-9. [PubMed:7116632 ]
  2. Kuhara T, Matsumoto I: Studies on the urinary acidic metabolites from three patients with methylmalonic aciduria. Biomed Mass Spectrom. 1980 Oct;7(10):424-8. [PubMed:6111361 ]
  3. Lehnert W, Junker A: [2-Methyl-3-oxovaleric acid: a characteristic metabolite in propionic acidemia]. Clin Chim Acta. 1980 May 21;104(1):47-51. [PubMed:7389125 ]
  4. DelValle JA, Merinero B, Jimenez A, Garcia MJ, Ugarte M, Omenaca F, Neustadt G, Quero J: Dietary treatment and biochemical studies on a neonatal case of propionyl-CoA carboxylase deficiency. J Inherit Metab Dis. 1982;5(2):121-4. [PubMed:6820422 ]
  5. Boebel KP, Baker DH: Comparative utilization of the alpha-keto and D- and L-alpha-hydroxy analogs of leucine, isoleucine and valine by chicks and rats. J Nutr. 1982 Oct;112(10):1929-39. [PubMed:7119896 ]
  6. Poston JM: Cobalamin-dependent formation of leucine and beta-leucine by rat and human tissue. Changes in pernicious anemia. J Biol Chem. 1980 Nov 10;255(21):10067-72. [PubMed:7430116 ]
  7. Stabler SP, Lindenbaum J, Allen RH: Failure to detect beta-leucine in human blood or leucine 2,3-aminomutase in rat liver using capillary gas chromatography-mass spectrometry. J Biol Chem. 1988 Apr 25;263(12):5581-8. [PubMed:3356699 ]
  8. Metz S, Holland S, Johnson L, Espling E, Rabaglia M, Segu V, Brockenbrough JS, Tran PO: Inosine-5'-monophosphate dehydrogenase is required for mitogenic competence of transformed pancreatic beta cells. Endocrinology. 2001 Jan;142(1):193-204. [PubMed:11145582 ]
  9. Brunk E, Sahoo S, Zielinski DC, Altunkaya A, Drager A, Mih N, Gatto F, Nilsson A, Preciat Gonzalez GA, Aurich MK, Prlic A, Sastry A, Danielsdottir AD, Heinken A, Noronha A, Rose PW, Burley SK, Fleming RMT, Nielsen J, Thiele I, Palsson BO: Recon3D enables a three-dimensional view of gene variation in human metabolism. Nat Biotechnol. 2018 Mar;36(3):272-281. doi: 10.1038/nbt.4072. Epub 2018 Feb 19. [PubMed:29457794 ]