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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:38 UTC
HMDB IDHMDB00408
Secondary Accession NumbersNone
Metabolite Identification
Common Name2-Methyl-3-ketovaleric acid
Description2-Methyl-3-ketovaleric acid is a metabolite of beta-leucine in the beta-keto pathway of leucine metabolism. 2-Methyl-3-ketovaleric acid is a known pathological metabolite and associated with propionic acidemia, especially during periods of ketoacidosis; a severe deficiency of propionyl-CoA carboxylase in cultured fibroblasts has been detected in some neonatal cases. (PMID 7389125 , 6820422 , 7119896 , 7430116 , 3356699 ). 2-Methyl-3-ketovaleric acid induces a strong activity of inosine-5'-monophosphate dehydrogenase (IMPDH; the rate-limiting enzyme in GTP synthesis), mimicking glucose. (PMID: 11145582 ).
Structure
Thumb
Synonyms
ValueSource
2-Methyl-3-ketovalerateHMDB
2-Methyl-3-oxo-pentanoateHMDB
2-Methyl-3-oxo-pentanoic acidHMDB
2-Methyl-3-oxovalerateHMDB
2-Methyl-3-oxovaleric acidHMDB
beta-KetoisocaproateHMDB
beta-Ketoisocaproic acidHMDB
Chemical FormulaC6H10O3
Average Molecular Weight130.1418
Monoisotopic Molecular Weight130.062994186
IUPAC Name2-methyl-3-oxopentanoic acid
Traditional Name2-methyl-3-oxovaleric acid
CAS Registry Number14925-93-0
SMILES
CCC(=O)C(C)C(O)=O
InChI Identifier
InChI=1S/C6H10O3/c1-3-5(7)4(2)6(8)9/h4H,3H2,1-2H3,(H,8,9)
InChI KeyInChIKey=QTLYPQZWYOHATR-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound 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
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Branched fatty acid
  • Beta-keto acid
  • Fatty acyl
  • 1,3-dicarbonyl compound
  • Beta-hydroxy ketone
  • Ketone
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected 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
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility30 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility60.6 mg/mLALOGPS
logP0.72ALOGPS
logP1.24ChemAxon
logS-0.33ALOGPS
pKa (Strongest Acidic)4.32ChemAxon
pKa (Strongest Basic)-7.6ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity31.74 m3·mol-1ChemAxon
Polarizability12.95 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-2900000000-b53ef30491933435a749View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-4900000000-3c46dc3d99a57de6904eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-001i-6900000000-ed7da1f432da0405f0b9View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
UrineDetected and Quantified<2 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.1(0.8-1.3) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothAutosomal dominant polycystic kidney disease (ADPKD) details
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022024
KNApSAcK IDNot Available
Chemspider ID164251
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00408
Metagene LinkHMDB00408
METLIN ID5397
PubChem Compound189028
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceHirai, Yasuhiro; Aida, Takuzo; Inoue, Shohei. Artificial photosynthesis of b-ketocarboxylic acids from carbon dioxide and ketones via enolate complexes of aluminum porphyrin. , Journal of the American Chemical Society (1989), 111(8), 3062-3.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Kuhara T, Shinka T, Matsuo M, Matsumoto I: Increased excretion of lactate, glutarate, 3-hydroxyisovalerate and 3-methylglutaconate during clinical episodes of propionic acidemia. Clin Chim Acta. 1982 Aug 4;123(1-2):101-9. [7116632 ]
  2. Kuhara T, Matsumoto I: Studies on the urinary acidic metabolites from three patients with methylmalonic aciduria. Biomed Mass Spectrom. 1980 Oct;7(10):424-8. [6111361 ]
  3. Lehnert W, Junker A: [2-Methyl-3-oxovaleric acid: a characteristic metabolite in propionic acidemia] Clin Chim Acta. 1980 May 21;104(1):47-51. [7389125 ]
  4. Stabler SP, Lindenbaum J, Allen RH: Failure to detect beta-leucine in human blood or leucine 2,3-aminomutase in rat liver using capillary gas chromatography-mass spectrometry. J Biol Chem. 1988 Apr 25;263(12):5581-8. [3356699 ]
  5. DelValle JA, Merinero B, Jimenez A, Garcia MJ, Ugarte M, Omenaca F, Neustadt G, Quero J: Dietary treatment and biochemical studies on a neonatal case of propionyl-CoA carboxylase deficiency. J Inherit Metab Dis. 1982;5(2):121-4. [6820422 ]
  6. Boebel KP, Baker DH: Comparative utilization of the alpha-keto and D- and L-alpha-hydroxy analogs of leucine, isoleucine and valine by chicks and rats. J Nutr. 1982 Oct;112(10):1929-39. [7119896 ]
  7. Poston JM: Cobalamin-dependent formation of leucine and beta-leucine by rat and human tissue. Changes in pernicious anemia. J Biol Chem. 1980 Nov 10;255(21):10067-72. [7430116 ]
  8. Metz S, Holland S, Johnson L, Espling E, Rabaglia M, Segu V, Brockenbrough JS, Tran PO: Inosine-5'-monophosphate dehydrogenase is required for mitogenic competence of transformed pancreatic beta cells. Endocrinology. 2001 Jan;142(1):193-204. [11145582 ]