Record Information
Version2.0
Creation Date2012-05-31 14:04:48 -0600
Update Date2015-06-03 15:54:43 -0600
Secondary Accession Numbers
  • ECMDB04092
Identification
Name:alpha-Ketoisovaleric acid
DescriptionAlpha-ketoisovaleric acid is a branched chain organic acid which is a precursor to leucine and valine synthesis. It is also a degradation product from valine. The enzyme dihydroxy-acid dehydratase catalyzes the fourth step in the biosynthesis of isoleucine and valine, through the dehydration of 2, 3-dihydroxy-isovaleic acid into alpha-ketoisovaleric acid.
Structure
Thumb
Synonyms:
  • α-keto-isovalerate
  • α-keto-isovaleric acid
  • α-keto-valine
  • α-ketoisopentanoate
  • α-ketoisopentanoic acid
  • α-ketovaline
  • α-oxoisovalerate
  • α-oxoisovaleric acid
  • 2-Keto-3-Methylbutyrate
  • 2-Keto-3-Methylbutyric acid
  • 2-Keto-isovalerate
  • 2-Keto-isovaleric acid
  • 2-Ketoisovalerate
  • 2-Ketoisovaleric acid
  • 2-Ketoisvalerate
  • 2-ketoisvaleric acid
  • 2-Ketovaline
  • 2-KIV
  • 2-Oxo-3-methyl-butyrate
  • 2-oxo-3-Methyl-butyric acid
  • 2-Oxo-3-methylbutanoate
  • 2-Oxo-3-methylbutanoic acid
  • 2-Oxo-3-methylbutyrate
  • 2-Oxo-3-methylbutyric acid
  • 2-Oxoisopentanoate
  • 2-Oxoisopentanoic acid
  • 2-Oxoisovalerate
  • 2-Oxoisovaleric acid
  • 3-Methyl-2-oxo-Butanoate
  • 3-Methyl-2-oxo-Butanoic acid
  • 3-Methyl-2-oxo-Butyrate
  • 3-Methyl-2-oxo-Butyric acid
  • 3-Methyl-2-oxobutanoate
  • 3-Methyl-2-oxobutanoic acid
  • 3-Methyl-2-oxobutinoate
  • 3-Methyl-2-oxobutinoic acid
  • 3-Methyl-2-oxobutyrate
  • 3-Methyl-2-oxobutyric acid
  • A-Keto-b-Methylbutyrate
  • A-Keto-b-Methylbutyric acid
  • A-Keto-Isovalerate
  • A-Keto-Isovaleric acid
  • a-keto-Valine
  • a-Ketoisopentanoate
  • a-Ketoisopentanoic acid
  • a-Ketoisovalerate
  • a-Ketoisovaleric acid
  • a-Ketovaline
  • A-Oxo-b-methylbutyrate
  • A-Oxo-b-methylbutyric acid
  • A-Oxoisovalerate
  • A-Oxoisovaleric acid
  • Alpha-Keto-beta-Methylbutyrate
  • Alpha-Keto-beta-Methylbutyric acid
  • Alpha-Keto-Isovalerate
  • Alpha-Keto-Isovaleric acid
  • Alpha-Keto-valine
  • Alpha-Ketoisopentanoate
  • Alpha-Ketoisopentanoic acid
  • Alpha-Ketoisovalerate
  • Alpha-Ketoisovaleric acid
  • Alpha-Ketovaline
  • Alpha-Oxo-beta-methylbutyrate
  • Alpha-Oxo-beta-methylbutyric acid
  • Alpha-Oxoisovalerate
  • Alpha-Oxoisovaleric acid
  • Dimethylpyruvate
  • Dimethylpyruvic acid
  • Isopropylglyoxylate
  • Isopropylglyoxylic acid
  • Ketovaline
  • α-keto-Isovalerate
  • α-keto-Isovaleric acid
  • α-keto-Valine
  • α-keto-β-Methylbutyrate
  • α-keto-β-Methylbutyric acid
  • α-Ketoisopentanoate
  • α-Ketoisopentanoic acid
  • α-Ketoisovalerate
  • α-Ketoisovaleric acid
  • α-Ketovaline
  • α-oxo-β-Methylbutyrate
  • α-oxo-β-Methylbutyric acid
  • α-Oxoisovalerate
  • α-Oxoisovaleric acid
Chemical Formula:C5H8O3
Weight:Average: 116.1152
Monoisotopic: 116.047344122
InChI Key:QHKABHOOEWYVLI-UHFFFAOYSA-N
InChI:InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)
CAS number:759-05-7
IUPAC Name:3-methyl-2-oxobutanoic acid
Traditional IUPAC Name:α-ketoisovalerate
SMILES:CC(C)C(=O)C(O)=O
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
Physical Properties
State:Solid
Charge:-1
Melting point:31.5 °C
Experimental Properties:
PropertyValueSource
Water Solubility:0.1 g/mL MeOH hot clearPhysProp
LogP:0.329PhysProp
Predicted Properties
PropertyValueSource
Water Solubility30.2 g/LALOGPS
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
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Biological Properties
Cellular Locations:Cytoplasm
Reactions:
SMPDB Pathways:
Pantothenate and CoA biosynthesisPW000828 ThumbThumb?image type=greyscaleThumb?image type=simple
KEGG Pathways:
  • Pantothenate and CoA biosynthesis ec00770
  • Valine, leucine and isoleucine biosynthesis ec00290
  • Valine, leucine and isoleucine degradation ec00280
EcoCyc Pathways:
Concentrations
Not Available
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
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, PositiveNot AvailableView in JSpectraViewer
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
LC-MS/MSLC-MS/MS Spectrum - 20V, Positivesplash10-00kf-9300000000-30d3bdfb6d38dbb8e101View in MoNA
LC-MS/MSLC-MS/MS Spectrum - 40V, Positivesplash10-000l-9000000000-879b6502ff380673b776View in MoNA
LC-MS/MSLC-MS/MS Spectrum - 10V, Positivesplash10-0159-9800000000-0cf4d3a0e7b3356995c6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - 35V, Positivesplash10-014o-9900000000-f35430a2eaea0d81df32View 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
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-01b9-6900000000-7f00871b074cbd9a42e6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-06di-9400000000-6d56778068e11e4f8e85View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000000000-b445e7fc67a8ff2bd3baView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-006x-9000000000-733fb7227d2d053b112fView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H 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
References
References:
  • Chuang DT, Niu WL, Cox RP: Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects. Biochem J. 1981 Oct 15;200(1):59-67. Pubmed: 6895847
  • Gallina DL, Dominguez JM, Hoschoian JC, Barrio JR: Maintenance of nitrogen balance in a young woman by substitution of -ketoisovaleric acid for valine. J Nutr. 1971 Sep;101(9):1165-7. Pubmed: 5096137
  • Kanehisa, M., Goto, S., Sato, Y., Furumichi, M., Tanabe, M. (2012). "KEGG for integration and interpretation of large-scale molecular data sets." Nucleic Acids Res 40:D109-D114. Pubmed: 22080510
  • Keseler, I. M., Collado-Vides, J., Santos-Zavaleta, A., Peralta-Gil, M., Gama-Castro, S., Muniz-Rascado, L., Bonavides-Martinez, C., Paley, S., Krummenacker, M., Altman, T., Kaipa, P., Spaulding, A., Pacheco, J., Latendresse, M., Fulcher, C., Sarker, M., Shearer, A. G., Mackie, A., Paulsen, I., Gunsalus, R. P., Karp, P. D. (2011). "EcoCyc: a comprehensive database of Escherichia coli biology." Nucleic Acids Res 39:D583-D590. Pubmed: 21097882
  • 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
  • Livesey G, Lund P: Binding of branched-chain 2-oxo acids to bovine serum albumin. Biochem J. 1982 Apr 15;204(1):265-72. Pubmed: 7115325
  • Schaefer K, von Herrath D, Erley CM, Asmus G: Calcium ketovaline as new therapy for uremic hyperphosphatemia. Miner Electrolyte Metab. 1990;16(6):362-4. Pubmed: 2089249
  • Schauder P, Schroder K, Langenbeck U: Serum branched-chain amino and keto acid response to a protein-rich meal in man. Ann Nutr Metab. 1984;28(6):350-6. Pubmed: 6393856
  • Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. Pubmed: 6422161
  • Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. Pubmed: 2026685
  • Tsuchiya H, Hashizume I, Tokunaga T, Tatsumi M, Takagi N, Hayashi T: High-performance liquid chromatography of alpha-keto acids in human saliva. Arch Oral Biol. 1983;28(11):989-92. Pubmed: 6581765
  • van der Werf, M. J., Overkamp, K. M., Muilwijk, B., Coulier, L., Hankemeier, T. (2007). "Microbial metabolomics: toward a platform with full metabolome coverage." Anal Biochem 370:17-25. Pubmed: 17765195
  • Winder, C. L., Dunn, W. B., Schuler, S., Broadhurst, D., Jarvis, R., Stephens, G. M., Goodacre, R. (2008). "Global metabolic profiling of Escherichia coli cultures: an evaluation of methods for quenching and extraction of intracellular metabolites." Anal Chem 80:2939-2948. Pubmed: 18331064
  • Yurtsever D. (2007). Fatty acid methyl ester profiling of Enterococcus and Esherichia coli for microbial source tracking. M.sc. Thesis. Villanova University: U.S.A
Synthesis Reference:Pirrung, 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
External Links:
ResourceLink
CHEBI ID16530
HMDB IDHMDB00019
Pubchem Compound ID49
Kegg IDC00141
ChemSpider ID48
Wikipedia IDAlpha-Ketoisovaleric acid
BioCyc ID2-KETO-ISOVALERATE
EcoCyc ID2-KETO-ISOVALERATE
Ligand ExpoKIV

Enzymes

General function:
Involved in metabolic process
Specific function:
2,3-dihydroxy-3-methylbutanoate = 3-methyl-2- oxobutanoate + H(2)O
Gene Name:
ilvD
Uniprot ID:
P05791
Molecular weight:
65531
Reactions
2,3-dihydroxy-3-methylbutanoate = 3-methyl-2-oxobutanoate + H(2)O.
General function:
Involved in transferase activity
Specific function:
L-valine + pyruvate = 3-methyl-2-oxobutanoate + L-alanine
Gene Name:
avtA
Uniprot ID:
P09053
Molecular weight:
46711
Reactions
L-valine + pyruvate = 3-methyl-2-oxobutanoate + L-alanine.
General function:
Involved in 2-isopropylmalate synthase activity
Specific function:
Catalyzes the condensation of the acetyl group of acetyl-CoA with 3-methyl-2-oxobutanoate (2-oxoisovalerate) to form 3-carboxy-3-hydroxy-4-methylpentanoate (2-isopropylmalate)
Gene Name:
leuA
Uniprot ID:
P09151
Molecular weight:
57297
Reactions
Acetyl-CoA + 3-methyl-2-oxobutanoate + H(2)O = (2S)-2-isopropylmalate + CoA.
General function:
Involved in 3-methyl-2-oxobutanoate hydroxymethyltransferase activity
Specific function:
Catalyzes the reversible reaction in which hydroxymethyl group from 5,10-methylenetetrahydrofolate is tranferred onto alpha-ketoisovalerate to form ketopantoate
Gene Name:
panB
Uniprot ID:
P31057
Molecular weight:
28237
Reactions
5,10-methylenetetrahydrofolate + 3-methyl-2-oxobutanoate + H(2)O = tetrahydrofolate + 2-dehydropantoate.
General function:
Involved in 1-aminocyclopropane-1-carboxylate synthase activity
Specific function:
Specific function unknown
Gene Name:
yfbQ
Uniprot ID:
P0A959
Molecular weight:
45517
Reactions
L-alanine + 2-oxoglutarate = pyruvate + L-glutamate.
General function:
Involved in catalytic activity
Specific function:
Acts on leucine, isoleucine and valine
Gene Name:
ilvE
Uniprot ID:
P0AB80
Molecular weight:
34093
Reactions
L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate.
L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate.
L-valine + 2-oxoglutarate = 3-methyl-2-oxobutanoate + L-glutamate.