Alpha-ketoisovaleric acid (CHEM003385)

Record Information
Version1.0
Creation Date2014-08-29 06:51:12 UTC
Update Date2026-05-14 18:02:09 UTC
Accession NumberCHEM003385
Identification
Common NameAlpha-ketoisovaleric acid
ClassSmall Molecule
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.
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
MOLSDFPDBSMILESInChI
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 Mass116.115 g/mol
Monoisotopic Mass116.047 g/mol
CAS Registry Number759-05-7
IUPAC Name3-methyl-2-oxobutanoic acid
Traditional Nameα-ketoisovalerate
SMILESCC(C)C(=O)C(O)=O
InChI IdentifierInChI=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
Biological Properties
StatusDetected and Not Quantified
OriginEndogenous
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid LocationsNot Available
Tissue Locations
  • Most Tissues
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 Point31.5°C
Boiling PointNot Available
SolubilityNot Available
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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectra
Toxicity Profile
Route of ExposureNot Available
Mechanism of ToxicityNot Available
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 alpha-ketoisovaleric acid are associated with Maple Syrup Urine Disease.
SymptomsNot Available
TreatmentNot Available
Concentrations
Not Available
External Links
DrugBank IDDB04074
HMDB IDHMDB0000019
FooDB IDFDB012250
Phenol Explorer IDNot Available
KNApSAcK IDC00007623
BiGG ID34011
BioCyc ID2-KETO-ISOVALERATE
METLIN ID5091
PDB IDNot Available
Wikipedia LinkAlpha-Ketoisovaleric acid
Chemspider ID48
ChEBI ID16530
PubChem Compound ID49
Kegg Compound IDC00141
YMDB IDYMDB00365
ECMDB IDECMDB00019
References
Synthesis ReferenceNot Available
MSDSLink
General References
1. 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.
2. 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.
3. 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.
4. 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.
5. Livesey G, Lund P: Binding of branched-chain 2-oxo acids to bovine serum albumin. Biochem J. 1982 Apr 15;204(1):265-72.
6. 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.
7. 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.
8. 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.
9. 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.
10. 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.
11. 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.
12. 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.
13. 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.
14. https://www.ncbi.nlm.nih.gov/pubmed/?term=17190852
15. https://www.ncbi.nlm.nih.gov/pubmed/?term=192504
16. https://www.ncbi.nlm.nih.gov/pubmed/?term=7021997