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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 17:47:27 UTC
HMDB IDHMDB0000552
Secondary Accession Numbers
  • HMDB00552
Metabolite Identification
Common Name3-Methylglutarylcarnitine
Description3-Methylglutarylcarnitine is a diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. It is also identified in the urine of patients with Reye like syndrome. (PMID 3958190 ; 10927963).
Structure
Thumb
Synonyms
ValueSource
Methylglutaryl-L-carnitineHMDB
Chemical FormulaC13H23NO6
Average Molecular Weight289.3248
Monoisotopic Molecular Weight289.152537473
IUPAC Name5-{[1-carboxy-3-(trimethylazaniumyl)propan-2-yl]oxy}-3-methyl-5-oxopentanoate
Traditional Name5-{[1-carboxy-3-(trimethylammonio)propan-2-yl]oxy}-3-methyl-5-oxopentanoate
CAS Registry Number102673-95-0
SMILES
CC(CC([O-])=O)CC(=O)OC(CC(O)=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C13H23NO6/c1-9(5-11(15)16)6-13(19)20-10(7-12(17)18)8-14(2,3)4/h9-10H,5-8H2,1-4H3,(H-,15,16,17,18)
InChI KeyHFCPFJNSBPQJDP-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentAcyl carnitines
Alternative Parents
Substituents
  • Acyl-carnitine
  • Tricarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic nitrogen compound
  • Organic salt
  • Organooxygen compound
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Cellular process:

    Chemical reaction:

    Biochemical process:

    Biochemical pathway:

Role

Industrial application:

Biological role:

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 Solubility0.066 g/LALOGPS
logP-1.6ALOGPS
logP-3.9ChemAxon
logS-3.7ALOGPS
pKa (Strongest Acidic)3.42ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area103.73 ŲChemAxon
Rotatable Bond Count10ChemAxon
Refractivity92.68 m³·mol⁻¹ChemAxon
Polarizability29.58 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9110000000-06a3954e2ebd1ad2d1c8View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Saliva
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    BloodDetected and Quantified0.020-0.060 uMAdult (>18 years old)BothNormal details
    SalivaDetected and Quantified0.088 +/- 0.011 uMAdult (>18 years old)BothNormal
      • Zerihun T. Dame, ...
    details
    UrineDetected and Quantified0.030 (0.016-0.052) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified0.049 (0.0327) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect 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 IDFDB022112
    KNApSAcK IDNot Available
    Chemspider ID113619
    KEGG Compound IDNot Available
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID5536
    PubChem Compound128145
    PDB IDNot Available
    ChEBI ID70857
    References
    Synthesis ReferenceJooste, S.; Erasmus, E.; Mienie, L. J.; de Wet, W. J.; Gibson, K. M. The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. Clinica Chimica Acta (1994), 230(1), 1-8.
    Material Safety Data Sheet (MSDS)Not Available
    General References
    1. Roe CR, Millington DS, Maltby DA: Identification of 3-methylglutarylcarnitine. A new diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. J Clin Invest. 1986 Apr;77(4):1391-4. [PubMed:3958190 ]
    2. Lee C, Tsai FJ, Wu JY, Peng CT, Tsai CH, Hwu WL, Wang TR, Millington DS: 3-hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case. Acta Paediatr Taiwan. 1999 Nov-Dec;40(6):445-7. [PubMed:10927963 ]
    3. Jooste S, Erasmus E, Mienie LJ, de Wet WJ, Gibson KM: The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. Clin Chim Acta. 1994 Oct 14;230(1):1-8. [PubMed:7850987 ]