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Record Information
Version1.0
Creation Date2016-09-30 22:18:31 UTC
Update Date2020-05-21 16:28:44 UTC
BMDB IDBMDB0000019
Secondary Accession Numbers
  • BMDB00019
Metabolite Identification
Common NameAlpha-ketoisovaleric acid
Descriptionalpha-Ketoisovaleric acid, also known as a-keto-isovalerate or 3-methyl-2-oxobutanoate, 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. alpha-Ketoisovaleric acid is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. alpha-Ketoisovaleric acid exists in all living species, ranging from bacteria to humans. alpha-Ketoisovaleric acid is a potentially toxic compound. alpha-Ketoisovaleric acid, with regard to humans, has been found to be associated with the diseases such as colorectal cancer; alpha-ketoisovaleric acid has also been linked to the inborn metabolic disorder maple syrup urine disease.
Structure
Thumb
Synonyms
ValueSource
2-Keto-3-methylbutyric acidChEBI
2-Ketoisovaleric acidChEBI
2-KetovalineChEBI
2-oxo-3-Methylbutanoic acidChEBI
2-oxo-3-Methylbutyric acidChEBI
2-Oxoisovaleric acidChEBI
3-Methyl-2-oxobutanoateChEBI
3-Methyl-2-oxobutyric acidChEBI
alpha-Keto-isovaleric acidChEBI
alpha-KetovalineChEBI
alpha-oxo-beta-MethylbutyricacidChEBI
alpha-Oxoisovaleric acidChEBI
Dimethylpyruvic acidChEBI
Isopropylglyoxylic acidChEBI
2-oxo-3-MethylbutanoateKegg
2-OxoisovalerateKegg
2-OxoisopentanoateKegg
2-Keto-3-methylbutyrateGenerator
2-KetoisovalerateGenerator
2-oxo-3-MethylbutyrateGenerator
3-Methyl-2-oxobutanoic acidGenerator
3-Methyl-2-oxobutyrateGenerator
a-Keto-isovalerateGenerator
a-Keto-isovaleric acidGenerator
alpha-Keto-isovalerateGenerator
Α-keto-isovalerateGenerator
Α-keto-isovaleric acidGenerator
a-KetovalineGenerator
Α-ketovalineGenerator
a-oxo-b-MethylbutyricacidGenerator
Α-oxo-β-methylbutyricacidGenerator
a-OxoisovalerateGenerator
a-Oxoisovaleric acidGenerator
alpha-OxoisovalerateGenerator
Α-oxoisovalerateGenerator
Α-oxoisovaleric acidGenerator
DimethylpyruvateGenerator
IsopropylglyoxylateGenerator
2-Oxoisopentanoic acidGenerator
a-KetoisovalerateGenerator
a-Ketoisovaleric acidGenerator
alpha-KetoisovalerateGenerator
Α-ketoisovalerateGenerator
Α-ketoisovaleric acidGenerator
2-Ketoisvaleric acidHMDB
2-oxo-3-Methyl-butyrateHMDB
3-Methyl-2-oxo-butanoateHMDB
3-Methyl-2-oxo-butanoic acidHMDB
3-Methyl-2-oxo-butyrateHMDB
3-Methyl-2-oxo-butyric acidHMDB
3-Methyl-2-oxobutinoateHMDB
3-Methyl-2-oxobutinoic acidHMDB
a-Keto-b-methylbutyrateHMDB
a-Keto-b-methylbutyric acidHMDB
a-oxo-b-MethylbutyrateHMDB
a-oxo-b-Methylbutyric acidHMDB
alpha-Keto-beta-methylbutyrateHMDB
alpha-Keto-beta-methylbutyric acidHMDB
alpha-oxo-beta-MethylbutyrateHMDB
alpha-oxo-beta-Methylbutyric acidHMDB
KetovalineHMDB
alpha-Ketoisopentanoic acidHMDB
alpha-Ketoisovaleric acidGenerator, MeSH
Chemical FormulaC5H8O3
Average Molecular Weight116.1152
Monoisotopic Molecular Weight116.047344122
IUPAC Name3-methyl-2-oxobutanoic acid
Traditional Nameα-ketoisovalerate
CAS Registry Number759-05-7
SMILES
CC(C)C(=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)
InChI KeyQHKABHOOEWYVLI-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
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Exogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cell membrane
  • Cytoplasm
  • Membrane
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point31.5 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
logP0.49ALOGPS
logP1.31ChemAxon
logS-0.59ALOGPS
pKa (Strongest Acidic)3.37ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.19 m³·mol⁻¹ChemAxon
Polarizability11.04 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) (Non-derivatized)splash10-000i-9500000000-ff936b879a69b5d118f8View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-8920000000-e37b37d64d43dcf763f0View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9400000000-e3995acc4818a98d0f48View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-0f79-9720000000-5d89487273e44ea61a68View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-000i-9700000000-209c737dcac7df2b198cView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0f79-5920000000-759a2f01b4f52767ade2View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9500000000-ff936b879a69b5d118f8View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-8920000000-e37b37d64d43dcf763f0View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-000i-9400000000-e3995acc4818a98d0f48View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0f79-9720000000-5d89487273e44ea61a68View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-7900000000-7e325567183fb56996d5View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0ufr-0930000000-be90b06add7135b4a539View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00dl-9000000000-52f9d8fc8386e4c512feView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-006x-9600000000-52aefdc997dac92c8459View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9000000000-10ab58a33e9ca7dbace0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-000i-9000000000-ad51ff01c94b6046ad64View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-014i-0900000000-9993174a7b1801b90ddbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00xr-9500000000-293818b81e0879b6feb2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-00di-9000000000-75058f27a2178b9cf121View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0006-9000000000-4c20af39e8ee009d5278View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-014i-0900000000-9993174a7b1801b90ddbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00xr-9500000000-1a58c6a6b4f5477dabddView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-75058f27a2178b9cf121View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-cc4465a47e663be66df5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , negativesplash10-00di-9100000000-19511890852fce513a02View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00r2-9400000000-21f8c3fae79161c82099View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dl-9000000000-5f25e41413738bb6b5d7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-801af00dea93fcfd637dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-01b9-8900000000-5185c7dfc72c25069904View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00xs-9200000000-7e2a275a65197f96f5d7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0600-9000000000-0013e0ff06f9896a1337View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cell membrane
  • Cytoplasm
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Mammary Gland
  • Muscle
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
Mammary GlandDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
MuscleDetected and Quantified0.38 +/- 0.09 umol/g tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
MuscleDetected and Quantified0.45 +/- 0.09 umol/g tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
MuscleDetected and Quantified0.26 +/- 0.10 umol/g tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
MuscleDetected and Quantified0.29 +/- 0.10 umol/g tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
PlacentaExpected but not QuantifiedNot ApplicableNot SpecifiedNot SpecifiedNormal
  • Not Applicable
details
Abnormal Concentrations
Not Available
HMDB IDHMDB0000019
DrugBank IDDB04074
Phenol Explorer Compound IDNot Available
FooDB IDFDB012250
KNApSAcK IDC00007623
Chemspider ID48
KEGG Compound IDC00141
BioCyc ID2-KETO-ISOVALERATE
BiGG ID34011
Wikipedia LinkAlpha-Ketoisovaleric acid
METLIN ID5091
PubChem Compound49
PDB IDNot Available
ChEBI ID16530
References
Synthesis ReferencePirrung, Michael C.; Ha, Hyun Joon; Holmes, Christopher P. Purification and inhibition of spinach a,b-dihydroxyacid dehydratase . Journal of Organic Chemistry (1989), 54(7), 1543-8.
Material Safety Data Sheet (MSDS)Not Available
General ReferencesNot Available

Enzymes

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
General function:
Energy production and conversion
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHA
Uniprot ID:
P11178
Molecular weight:
51678.0
Reactions
Alpha-ketoisovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxidedetails
General function:
Energy production and conversion
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHB
Uniprot ID:
P21839
Molecular weight:
42935.0
Reactions
Alpha-ketoisovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxidedetails