Hmp_logo

Human Metabolome Database Version 3.5

HMDB has recently undergone some major changes, if you are experiencing problems please click here to provide us with feedback.

Showing metabocard for Alpha-ketoisovaleric acid (HMDB00019)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:45 -0700
HMDB ID HMDB00019
Secondary Accession Numbers
  • HMDB04260
Metabolite Identification
Common Name Alpha-ketoisovaleric acid
Description Alpha-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
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 2-Keto-3-Methylbutyrate
  2. 2-Keto-3-Methylbutyric acid
  3. 2-Ketoisovalerate
  4. 2-Ketoisovaleric acid
  5. 2-Ketoisvaleric acid
  6. 2-Oxo-3-methyl-butyrate
  7. 2-Oxo-3-methylbutanoate
  8. 2-Oxo-3-methylbutanoic acid
  9. 2-Oxo-3-methylbutyrate
  10. 2-Oxo-3-methylbutyric acid
  11. 2-Oxoisovalerate
  12. 2-Oxoisovaleric acid
  13. 3-Methyl-2-oxo-Butanoate
  14. 3-Methyl-2-oxo-Butanoic acid
  15. 3-Methyl-2-oxo-Butyrate
  16. 3-Methyl-2-oxo-Butyric acid
  17. 3-Methyl-2-oxobutanoate
  18. 3-Methyl-2-oxobutanoic acid
  19. 3-Methyl-2-oxobutinoate
  20. 3-Methyl-2-oxobutinoic acid
  21. 3-Methyl-2-oxobutyrate
  22. 3-Methyl-2-oxobutyric acid
  23. a-Keto-b-Methylbutyrate
  24. a-Keto-b-Methylbutyric acid
  25. a-Keto-Isovalerate
  26. a-Keto-Isovaleric acid
  27. a-Ketoisovalerate
  28. a-Ketoisovaleric acid
  29. a-Oxo-b-methylbutyrate
  30. a-Oxo-b-methylbutyric acid
  31. a-Oxoisovalerate
  32. a-Oxoisovaleric acid
  33. alpha-Keto-beta-Methylbutyrate
  34. alpha-Keto-beta-Methylbutyric acid
  35. alpha-Keto-Isovalerate
  36. alpha-Keto-Isovaleric acid
  37. alpha-Ketoisovalerate
  38. alpha-Ketoisovaleric acid
  39. alpha-Oxo-beta-methylbutyrate
  40. alpha-Oxo-beta-methylbutyric acid
  41. alpha-Oxoisovalerate
  42. alpha-Oxoisovaleric acid
  43. Dimethylpyruvate
  44. Dimethylpyruvic acid
  45. Isopropylglyoxylate
  46. Isopropylglyoxylic acid
  47. Ketovaline
Chemical Formula C5H8O3
Average Molecular Weight 116.1152
Monoisotopic Molecular Weight 116.047344122
IUPAC Name 3-methyl-2-oxobutanoic acid
Traditional IUPAC Name α-ketoisovalerate
CAS Registry Number 759-05-7
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 Key QHKABHOOEWYVLI-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Lipids
Class Fatty Acids and Conjugates
Sub Class Branched Fatty Acids
Other Descriptors
  • 2-oxo monocarboxylic acid(ChEBI)
  • Aliphatic Acyclic Compounds
  • Branched fatty acids(KEGG)
  • Branched fatty acids(Lipidmaps)
  • Organic Compounds
  • short-chain fatty acid(ChEBI)
  • straight-chain saturated fatty acid(ChEBI)
Substituents
  • Alpha Keto Acid
  • Carboxylic Acid
  • Ketone
  • Short Chain Keto Acid
Direct Parent Branched Fatty Acids
Ontology
Status Detected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 31.5 °C Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 30.2 g/L ALOGPS
LogP 0.49 ALOGPS
LogP 1.31 ChemAxon
LogS -0.59 ALOGPS
pKa (strongest acidic) 3.37 ChemAxon
pKa (strongest basic) -9.7 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 54.37 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 27.19 ChemAxon
Polarizability 11.04 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
[1H,13C] 2D NMR Spectrum
[1H,1H] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Most Tissues
Pathways
Name SMPDB Link KEGG Link
Valine, Leucine and Isoleucine Degradation SMP00032 map00280 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
14.0 (0.0-28.0) uM Children (1-13 year old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
11.0 +/- 1.7 uM Adult (>18 years old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
6 +/- 3 uM Not Specified Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
8.2 +/- 6.8 uM Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
Article_icon
0.16 +/- 0.48 umol/mmol creatinine Infant (0-1 year old) Both Normal Not Available
Urine Detected and Quantified
Article_icon
0.5 (0.2-1.1) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR
Urine Detected and Quantified
Article_icon
0.13 (0.00-0.54) umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and Quantified
Article_icon
0.07 (0.01-0.45) umol/mmol creatinine Adult (>18 years old) Female Normal Not Available
Urine Detected and Quantified
Article_icon
0.07 (0.01-0.45) umol/mmol creatinine Adult (>18 years old) Female Normal Not Available
Urine Detected and Quantified
Article_icon
1.00 (0.00-2.00) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
10.7 +/- 5.5 uM Adult (>18 years old) Not Specified Heart Transplant Not Available
Urine Detected and Quantified
Article_icon
550.00 (300.00-800.00) umol/mmol creatinine Adult (>18 years old) Both Maple syrup urine disease Not Available
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
    • http://www.metagene.de/program/d.prg?mp=MAPLE%20SYRUP%20URINE%20DISEASE
    Associated OMIM IDs
    • 248600 Link_out (Maple syrup urine disease)
    DrugBank ID DB04074 Link_out
    Phenol Explorer Compound ID Not Available
    Phenol Explorer Metabolite ID Not Available
    FoodDB ID FDB012250
    KNApSAcK ID C00007623 Link_out
    Chemspider ID 48 Link_out
    KEGG Compound ID C00141 Link_out
    BioCyc ID 2-KETO-ISOVALERATE Link_out
    BiGG ID 34011 Link_out
    Wikipedia Link Not Available
    NuGOwiki Link HMDB00019 Link_out
    Metagene Link HMDB00019 Link_out
    METLIN ID 5091 Link_out
    PubChem Compound 49 Link_out
    PDB ID KIV Link_out
    ChEBI ID 16530 Link_out
    References
    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) Download (PDF)
    General References
    1. 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 Link_out
    2. 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 Link_out
    3. 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 Link_out
    4. 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 Link_out
    5. 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 Link_out
    6. 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 Link_out
    7. 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 Link_out
    8. 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 Link_out
    9. 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 Link_out
    Enzymes
    Name: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
    Reactions:
    • pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2 [RN:R01699]
    Gene Name: PDHB
    Uniprot ID: P11177 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
    Reactions:
    • pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2 [RN:R01699]
    Gene Name: PDHA1
    Uniprot ID: P08559 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
    Reactions:
    • pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2 [RN:R01699]
    Gene Name: PDHA2
    Uniprot ID: P29803 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
    Reactions:
    • acetyl-CoA + enzyme N6-(dihydrolipoyl)lysine = CoA + enzyme N6-(S-acetyldihydrolipoyl)lysine [RN:R02569]
    Gene Name: DLAT
    Uniprot ID: P10515 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
    Reactions:
    • 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701]
    Gene Name: BCKDHB
    Uniprot ID: P21953 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
    Reactions:
    • 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701]
    Gene Name: BCKDHA
    Uniprot ID: P12694 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Branched-chain-amino-acid aminotransferase, cytosolic
    Reactions:
    • L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate [RN:R01090]
    Gene Name: BCAT1
    Uniprot ID: P54687 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Branched-chain-amino-acid aminotransferase, mitochondrial
    Reactions:
    • L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate [RN:R01090]
    Gene Name: BCAT2
    Uniprot ID: O15382 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Transporters
    Name: Monocarboxylate transporter 4
    Reactions:
    • --- []
    Gene Name: SLC16A3
    Uniprot ID: O15427 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Monocarboxylate transporter 1
    Reactions:
    • --- []
    Gene Name: SLC16A1
    Uniprot ID: P53985 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Monocarboxylate transporter 2
    Reactions:
    • --- []
    Gene Name: SLC16A7
    Uniprot ID: O60669 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA