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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:55 UTC
HMDB IDHMDB00552
Secondary Accession NumbersNone
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 KeyInChIKey=HFCPFJNSBPQJDP-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassTricarboxylic acids and derivatives
Direct ParentTricarboxylic acids and derivatives
Alternative Parents
Substituents
  • Tricarboxylic acid or derivatives
  • Acyl choline
  • Fatty acid ester
  • Choline
  • Fatty acyl
  • Quaternary ammonium salt
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organic salt
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Organic zwitterion
  • 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
  • Lipid catabolism, Fatty acid transport, Energy production
  • 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 SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.066 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count10ChemAxon
Refractivity92.68 m3·mol-1ChemAxon
Polarizability29.62 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Saliva
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
Normal
  • Not Applicable
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
Not Available
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
NuGOwiki LinkHMDB00552
Metagene LinkHMDB00552
METLIN ID5536
PubChem Compound128145
PDB IDNot Available
ChEBI IDNot Available
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. [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. [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. [7850987 ]