Np mrd loader

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Record Information
Version1.0
Created at2005-11-16 15:48:42 UTC
Updated at2020-11-24 22:14:08 UTC
NP-MRD IDNP0000265
Secondary Accession NumbersNone
Natural Product 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-oxo-pentanoic acidChEBI
2-Methyl-3-oxovaleric acidChEBI
beta-Ketoisocaproic acidChEBI
2-Methyl-3-oxo-pentanoateGenerator
2-Methyl-3-oxovalerateGenerator
b-KetoisocaproateGenerator
b-Ketoisocaproic acidGenerator
beta-KetoisocaproateGenerator
Β-ketoisocaproateGenerator
Β-ketoisocaproic acidGenerator
2-Methyl-3-ketovalerateGenerator
Chemical FormulaC6H10O3
Average Mass130.1418 Da
Monoisotopic Mass130.06299 Da
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 KeyQTLYPQZWYOHATR-UHFFFAOYSA-N
Spectra
Spectrum TypeDescriptionDepositor IDDeposition DateView
1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)Wishart Lab2021-06-20View Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)Wishart Lab2021-06-20View Spectrum
Species
Species of Origin
  • Animalia
  • Species Where Detected
    Species NameSourceReference
    Homo sapiens (Urine)KNApSAcK Database
    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
    • Beta-keto acid
    • Branched fatty acid
    • Methyl-branched fatty acid
    • Short-chain keto acid
    • Beta-hydroxy ketone
    • Fatty acyl
    • 1,3-dicarbonyl compound
    • 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
    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 g/LALOGPS
    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 ŲChemAxon
    Rotatable Bond Count3ChemAxon
    Refractivity31.74 m³·mol⁻¹ChemAxon
    Polarizability12.95 ųChemAxon
    Number of Rings0ChemAxon
    BioavailabilityYesChemAxon
    Rule of FiveYesChemAxon
    Ghose FilterNoChemAxon
    Veber's RuleNoChemAxon
    MDDR-like RuleNoChemAxon
    HMDB IDHMDB0000408
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB022024
    KNApSAcK IDC00052124
    Chemspider ID164251
    KEGG Compound IDNot Available
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID5397
    PubChem Compound189028
    PDB IDNot Available
    ChEBI ID86365
    Good Scents IDNot Available
    References
    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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:11145582 ]