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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:50 UTC
HMDB IDHMDB0000552
Secondary Accession Numbers
  • HMDB00552
Metabolite Identification
Common Name3-Methylglutarylcarnitine
Description
Structure
Thumb
Synonyms
ValueSource
(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acidHMDB
MethylglutarylcarnitineHMDB
O-3-MethylglutarylcarnitineHMDB
3-MethylglutarylcarnitineHMDB
Chemical FormulaC13H23NO6
Average Molecular Weight289.328
Monoisotopic Molecular Weight289.152537465
IUPAC Name(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Name(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylammonio)butanoate
CAS Registry Number102673-95-0
SMILES
C[C@H](CC(O)=O)CC(=O)O[C@H](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)/t9-,10-/m1/s1
InChI KeyHFCPFJNSBPQJDP-NXEZZACHSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acid esters
Direct ParentAcyl carnitines
Alternative Parents
Substituents
  • Acyl-carnitine
  • Tricarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organic salt
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect
Disposition
Biological locationRoute of exposureSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.76 uMNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
BloodDetected and Quantified0.049 (0.0327) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
Associated Disorders and Diseases
Disease References
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
Associated OMIM IDs
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022112
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound156907977
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00029840
Good Scents 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