3-Methyl-2-oxovaleric acid (CHEM003384)

IdentificationTaxonomyBiological PropertiesPhysical PropertiesToxicity ProfileSpectraConcentrationsLinksReferencesXML
Record Information
Version1.0
Creation Date2014-08-29 06:51:12 UTC
Update Date2026-04-14 20:28:24 UTC
Accession NumberCHEM003384
Identification
Common Name3-Methyl-2-oxovaleric acid
ClassSmall Molecule
Description3-Methyl-2-oxovaleric acid is a metabolite of isoleucine in man, animals and bacteria. It is the alpha-keto acid analogue of isoleucine. 3-Methyl-2-oxovaleric acid is produced from isoleucine by cytosolic branched chain aminotransferase 1 (EC:2.6.1.42), whereupon it is further degraded by branched chain keto acid dehydrogenase E1 to 2-Methyl-1-hydroxybutyl-ThPP. It is used as a clinical marker for maple syrup urine disease (MSUD). MSUD is caused by a deficiency of the branched-chain -keto acid dehydrogenase compex resulting in an accumulation of branched-chain amino acids and the corresponding -keto-and -hydroxy acids in blood, urine and cerebrospinal fluid causing neurological damage and mental retardation.
Contaminant Sources
  • EAFUS Chemicals
  • FooDB Chemicals
  • HMDB Contaminants - Feces
  • HMDB Contaminants - Urine
  • T3DB toxins
Contaminant Type
  • Animal Toxin
  • Food Toxin
  • Metabolite
  • Natural Compound
  • Organic Compound
Chemical Structure
Thumb
MOLSDFPDBSMILESInChI
Synonyms
ValueSource
2-oxo-3-Methyl-N-valeric acidChEBI
2-oxo-3-Methylpentanoic acidChEBI
2-oxo-3-MethylvalerateChEBI
2-oxo-3-Methylvaleric acidChEBI
2-OxoisoleucineChEBI
2-Oxokolavenic acidChEBI
3-Ethyl-3-methylpyruvic acidChEBI
3-Methyl-2-oxopentanoic acidChEBI
alpha-Keto-beta-methyl-N-valeric acidChEBI
alpha-Keto-beta-methylvaleric acidChEBI
alpha-oxo-beta-Methyl-N-valeric acidChEBI
alpha-oxo-beta-Methylvaleric acidChEBI
KetoisoleucineChEBI
2-oxo-3-Methyl-N-valerateGenerator
2-oxo-3-MethylpentanoateGenerator
2-OxokolavenateGenerator
3-Ethyl-3-methylpyruvateGenerator
3-Methyl-2-oxopentanoateGenerator
a-Keto-b-methyl-N-valerateGenerator
a-Keto-b-methyl-N-valeric acidGenerator
alpha-Keto-beta-methyl-N-valerateGenerator
Α-keto-β-methyl-N-valerateGenerator
Α-keto-β-methyl-N-valeric acidGenerator
a-Keto-b-methylvalerateGenerator
a-Keto-b-methylvaleric acidGenerator
alpha-Keto-beta-methylvalerateGenerator
Α-keto-β-methylvalerateGenerator
Α-keto-β-methylvaleric acidGenerator
a-oxo-b-Methyl-N-valerateGenerator
a-oxo-b-Methyl-N-valeric acidGenerator
alpha-oxo-beta-Methyl-N-valerateGenerator
Α-oxo-β-methyl-N-valerateGenerator
Α-oxo-β-methyl-N-valeric acidGenerator
a-oxo-b-MethylvalerateGenerator
a-oxo-b-Methylvaleric acidGenerator
alpha-oxo-beta-MethylvalerateGenerator
Α-oxo-β-methylvalerateGenerator
Α-oxo-β-methylvaleric acidGenerator
3-Methyl-2-oxovalerateGenerator
(3R)-3-Methyl-2-oxopentanoateHMDB
(3R)-3-Methyl-2-oxopentanoic acidHMDB
(R)-3-Methyl-2-oxopentanoateHMDB
(R)-3-Methyl-2-oxopentanoic acidHMDB
(S)-3-Methyl-2-oxopentanoateHMDB
(S)-3-Methyl-2-oxopentanoic acidHMDB
2-Keto-3-methylvalerateHMDB
2-Keto-3-methylvaleric acidHMDB
3-Methyl-2-oxo-pentanoateHMDB
3-Methyl-2-oxo-pentanoic acidHMDB
3-Methyl-2-oxo-valerateHMDB
3-Methyl-2-oxo-valeric acidHMDB
3-Methyl-2-oxovalericHMDB
2-Keto methylvalerateHMDB
KMVAHMDB
alpha-Keto-beta-methylvaleric acid, calcium salt, (S)-isomerHMDB
alpha-Keto-beta-methylvaleric acid, monosodium salt, (S)-isomerHMDB
alpha-Keto-beta-methylvaleric acid, (S)-isomerHMDB
alpha-Keto-beta-methylvaleric acid, calcium saltHMDB
alpha-Keto-beta-methylvaleric acid, sodium salt, (+-)-isomerHMDB
alpha-Keto-beta-methylvaleric acid, (+-)-isomerHMDB
alpha-Keto-beta-methylvaleric acid, sodium saltHMDB
Chemical FormulaC6H10O3
Average Molecular Mass130.142 g/mol
Monoisotopic Mass130.063 g/mol
CAS Registry Number1460-34-0
IUPAC Name3-methyl-2-oxopentanoic acid
Traditional Name3-methyl-2-oxopentanoic acid
SMILESCCC(C)C(=O)C(O)=O
InChI IdentifierInChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)
InChI KeyJVQYSWDUAOAHFM-UHFFFAOYSA-N
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
Substituents
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Alpha-keto acid
  • Fatty acyl
  • Alpha-hydroxy ketone
  • 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 Properties
StatusDetected and Not Quantified
OriginEndogenous
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid LocationsNot Available
Tissue Locations
  • Prostate
Pathways
NameSMPDB LinkKEGG Link
Valine, Leucine and Isoleucine DegradationSMP00032 map00280
Maple Syrup Urine DiseaseSMP00199 Not Available
ApplicationsNot Available
Biological Roles
Chemical RolesNot Available
Physical Properties
StateSolid
AppearanceWhite powder.
Experimental Properties
PropertyValue
Melting PointNot Available
Boiling PointNot Available
Solubility915.5 mg/mL
Predicted Properties
PropertyValueSource
Water Solubility9.86 g/LALOGPS
logP1ALOGPS
logP1.75ChemAxon
logS-1.1ALOGPS
pKa (Strongest Acidic)3.52ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity31.79 m³·mol⁻¹ChemAxon
Polarizability13.09 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-000i-9610000000-d2ffd4c2796688c555eeSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-000i-9720000000-9cbc934e100bedd87f17Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-9720000000-5acbdc73aee24c023763Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-6930000000-8c2cf1d8df538010611bSpectrum
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-000i-6930000000-7bef6751c4c607393e5eSpectrum
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0hg9-4950000000-3e1c700a6f553bf63afbSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9610000000-d2ffd4c2796688c555eeSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9720000000-9cbc934e100bedd87f17Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9720000000-5acbdc73aee24c023763Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-6930000000-8c2cf1d8df538010611bSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-056r-9000000000-d08073312b337cdae9e6Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-05bo-9400000000-9937bff65302162dc3b9Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000w-9300000000-0a6ff60455403c38be39Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0002-9000000000-7ee6d2fb63c6ea4cccfaSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-002b-9000000000-47d18dff23b741029581Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-004i-0900000000-ae9da2a9acfa56fc6091Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , negativesplash10-004r-5900000000-a3f740a5c859439255beSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-06sr-7900000000-4920c1aefcca814ec1f6Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0a4r-9100000000-16995fa8eb603d828a2fSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-a6ccc899039a438533aeSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004r-5900000000-50107933abbe9c175553Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01ri-9700000000-23998218af9ee2898594Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000000000-a30461e2df45eeb8f795Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Toxicity Profile
Route of ExposureNot Available
Mechanism of Toxicity3-Methyl-2-oxovaleric acid is produced from isoleucine by cytosolic branched chain aminotransferase 1, whereupon it is further degraded by branched chain keto acid dehydrogenase E1 to 2-methyl-1-hydroxybutyl-ThPP. It is used as a clinical marker for maple syrup urine disease (MSUD). MSUD is caused by a deficiency of the branched-chain -keto acid dehydrogenase complex resulting in an accumulation of branched-chain amino acids and the corresponding -keto-and -hydroxy acids in blood, urine and cerebrospinal fluid causing neurological damage and mental retardation.
MetabolismNot Available
Toxicity ValuesNot Available
Lethal DoseNot Available
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Uses/SourcesThis is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.
Minimum Risk LevelNot Available
Health EffectsChronically high levels of 3-methyl-2-oxovaleric acid are associated with Maple Syrup Urine Disease.
SymptomsNot Available
TreatmentNot Available
Concentrations
Not Available
DrugBank IDNot Available
HMDB IDHMDB0000491
FooDB IDFDB021447
Phenol Explorer IDNot Available
KNApSAcK IDNot Available
BiGG ID35662
BioCyc IDNot Available
METLIN ID5478
PDB IDNot Available
Wikipedia LinkNot Available
Chemspider ID46
ChEBI ID35932
PubChem Compound ID47
Kegg Compound IDC03465
YMDB IDNot Available
ECMDB IDECMDB00491
References
Synthesis ReferenceKondo, Shigeo; Sudo, Tetsuji; Ogiwara, Mitsuo; Takeuchi, Hiroshi. a-Oxo-b-methyl-n-valeric acid and its salts. Jpn. Kokai Tokkyo Koho (1979), 3 pp.
MSDSLink
General References
1. Kondo, Shigeo; Sudo, Tetsuji; Ogiwara, Mitsuo; Takeuchi, Hiroshi. a-Oxo-b-methyl-n-valeric acid and its salts. Jpn. Kokai Tokkyo Koho (1979), 3 pp.
2. Kondo, Shigeo; Sudo, Tetsuji; Ogiwara, Mitsuo; Takeuchi, Hiroshi. a-Oxo-b-methyl-n-valeric acid and its salts. Jpn. Kokai Tokkyo Koho (1979), 3 pp.
3. Wendel U, Even G, Langenbeck U, Schadewaldt P, Hummel W: Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease. Clin Chim Acta. 1992 Jun 15;208(1-2):85-91.
4. Przyrembel H, Bremer HJ, Duran M, Bruinvis L, Ketting D, Wadman SK, Baumgartner R, Irle U, Bachmann C: Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels. Eur J Pediatr. 1979 Jan 18;130(1):1-14.
5. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762.
6. Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S.
7. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043.
8. https://www.ncbi.nlm.nih.gov/pubmed/?term=1638756
9. https://www.ncbi.nlm.nih.gov/pubmed/?term=17190852
10. https://www.ncbi.nlm.nih.gov/pubmed/?term=759179