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Record Information
Version1.0
Creation Date2016-09-30 22:18:31 UTC
Update Date2020-06-04 20:27:53 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 NameNot Available
Traditional NameNot Available
CAS Registry Number759-05-7
SMILESNot Available
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 PropertiesNot Available
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Biological Properties
Cellular Locations
  • Cell membrane
  • Cytoplasm
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Mammary Gland
  • Muscle
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
Mammary GlandDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
MuscleDetected and Quantified380 +/- 20 nmol/g of tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
MuscleDetected and Quantified450 +/- 30 nmol/g of tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
MuscleDetected and Quantified260 +/- 20 nmol/g of tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
MuscleDetected and Quantified290 +/- 20 nmol/g of tissueNot SpecifiedNot Specified
Normal
    • S. F. Graham, T. ...
details
PlacentaExpected but not QuantifiedNot QuantifiedNot 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