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Showing metabocard for 2-Methylacetoacetic acid (HMDB03771)

IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesXML
Record Information
Version3.6
Creation Date2006-08-13 04:36:31 UTC
Update Date2017-03-02 21:27:22 UTC
HMDB IDHMDB03771
Secondary Accession NumbersNone
Metabolite Identification
Common Name2-Methylacetoacetic acid
Description2-Methylacetoacetic acid is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase (EC 2.3.1.9) deficiency (OMIM 607809 , ACAT1; OMIM 100678 , ACAT2; OMIM 604770 , ACAA2; OMIM 607809 ). Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1) , or the peroxisomes(ACAA2). Thiolases are CoA-dependent enzymes which catalyze the formation of a carbon-carbon bond in a Claisen condensation step and its reverse reaction via a thiolytic degradation mechanism. mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (T2) is important in the pathways for the synthesis and degradation of ketone bodies as well as for the degradation of 2-methylacetoacetyl-CoA. (PMID: 17371050 , 14518824 , 9744475 , 9001814 ).
Structure
Thumb
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
Synonyms
ValueSource
2-Methyl-3-oxo-butyric acidChEBI
2-Methyl-3-oxo-butyrateGenerator
2-MethylacetoacetateGenerator
(+/-)-2-methyl-3-oxobutanoateHMDB
(+/-)-2-methyl-3-oxobutanoic acidHMDB
2-Acetylpropionic acidHMDB
2-Methyl-3-ketobutyric acidHMDB
2-Methyl-3-oxo-butanoateHMDB
2-Methyl-3-oxo-butanoic acidHMDB
2-Methyl-3-oxobutanoateHMDB
2-Methyl-3-oxobutanoic acidHMDB
2-Methyl-3-oxobutyric acidHMDB
2-Methyl-acetoacetic acidHMDB
3-oxo-2-Methylbutyric acidHMDB
a-Methylacetoacetic acidHMDB
alpha-MethylacetoacetateHMDB
alpha-Methylacetoacetic acidHMDB
Chemical FormulaC5H8O3
Average Molecular Weight116.1152
Monoisotopic Molecular Weight116.047344122
IUPAC Name2-methyl-3-oxobutanoic acid
Traditional Nameacetopropionic acid
CAS Registry Number2382-59-4
SMILES
CC(C(C)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8)
InChI KeyGCXJINGJZAOJHR-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassKeto 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
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
  • Extracellular
  • Membrane
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility94.9 mg/mLALOGPS
logP0.2ALOGPS
logP0.54ChemAxon
logS-0.09ALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)-7.5ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.11 m3·mol-1ChemAxon
Polarizability11.09 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    		 details
    UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified<2 umol/mmol creatinineChildren (1 - 18 years old)Both
    Normal
      • BC Children's Hos...
    		 details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    UrineDetected and Quantified325.0 (0.0-650.0) umol/mmol creatinineChildren (1-13 years old)BothBeta-Ketothiolase deficiency details
    Associated Disorders and Diseases
    Disease References
    Beta-ketothiolase deficiency
    1. MetaGene [Link]
    Associated OMIM IDs
    • 203750 (Beta-ketothiolase deficiency)
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB023226
    KNApSAcK IDNot Available
    Chemspider ID133090
    KEGG Compound IDNot Available
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    NuGOwiki LinkHMDB03771
    Metagene LinkHMDB03771
    METLIN IDNot Available
    PubChem Compound150996
    PDB IDNot Available
    ChEBI ID37079
    References
    Synthesis ReferenceMiddleton B; Bartlett K The synthesis and characterisation of 2-methylacetoacetyl coenzyme A and its use in the identification of the site of the defect in 2-methylacetoacetic and 2-methyl-3-hydroxybutyric aciduria. Clinica chimica acta; international journal of clinical chemistry (1983), 128(2-3), 291-305.
    Material Safety Data Sheet (MSDS)Not Available
    General References
    1. Haapalainen AM, Merilainen G, Pirila PL, Kondo N, Fukao T, Wierenga RK: Crystallographic and kinetic studies of human mitochondrial acetoacetyl-CoA thiolase: the importance of potassium and chloride ions for its structure and function. Biochemistry. 2007 Apr 10;46(14):4305-21. Epub 2007 Mar 20. [PubMed:17371050 ]
    2. Fukao T, Nakamura H, Song XQ, Nakamura K, Orii KE, Kohno Y, Kano M, Yamaguchi S, Hashimoto T, Orii T, Kondo N: Characterization of N93S, I312T, and A333P missense mutations in two Japanese families with mitochondrial acetoacetyl-CoA thiolase deficiency. Hum Mutat. 1998;12(4):245-54. [PubMed:9744475 ]
    3. Fukao T, Zhang GX, Sakura N, Kubo T, Yamaga H, Hazama A, Kohno Y, Matsuo N, Kondo M, Yamaguchi S, Shigematsu Y, Kondo N: The mitochondrial acetoacetyl-CoA thiolase (T2) deficiency in Japanese patients: urinary organic acid and blood acylcarnitine profiles under stable conditions have subtle abnormalities in T2-deficient patients with some residual T2 activity. J Inherit Metab Dis. 2003;26(5):423-31. [PubMed:14518824 ]
    4. Fukao T, Kodama A, Aoyanagi N, Tsukino R, Uemura S, Song XQ, Watanebe H, Kuhara T, Matsumoto I, Orii T, Kondo N: Mild form of beta-ketothiolase deficiency (mitochondrial acetoacetyl-CoA thiolase deficiency) in two Japanese siblings: identification of detectable residual activity and cross-reactive material in EB-transformed lymphocytes. Clin Genet. 1996 Oct;50(4):263-6. [PubMed:9001814 ]