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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2007-05-23 10:45:07 UTC
Update Date2018-05-20 19:10:58 UTC
HMDB IDHMDB0006469
Secondary Accession Numbers
  • HMDB0013212
  • HMDB06469
  • HMDB13212
Metabolite Identification
Common NameLinoleyl carnitine
DescriptionLinoleyl carnitine is a long-chain acyl fatty acid derivative ester of carnitine. Long-chain acyl fatty acid derivatives accumulate in the cytosol and serum of patients suffering of mitochondrial carnitine palmitoyltransferase II (CPT II, EC 2.3.1.12) deficiency, the most common inherited disorder of lipid metabolism in adults. Carnitine palmitoyltransferase II deficiency is an autosomal recessive disorder of fatty acid metabolism presenting as two clinical phenotypes: (i) a severe infantile hepatocardiomuscular form and (ii) a milder adult muscular form. Energy production from long-chain fatty acids (LCFAs) requires the transport of LCFAs into the mitochondrial matrix. This transport is carnitine-dependent and involves translocation machinery. Mitochondrial fatty acid oxidation disorders cause hypoglycaemia, hepatic dysfunction, myopathy, cardiomyopathy and encephalopathy. Patients with end-stage renal disease (ESRD) undergoing long-term haemodialysis exhibit elevated acylcarnitine concentrations (PMID: 11999976 , 10682306 , 15025677 , 16168195 ). Moreover, linoleyl carnitine is found to be associated with glutaric aciduria II, which is an inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
DecadienylcarnitineChEBI
Octadecadienyl-L-carnitineChEBI
3-Carboxy-N,N,N-trimethyl-2-[(1-oxo-9,12-octadecadienyl)oxy]-1-propanaminiumHMDB
3-Carboxy-N,N,N-trimethyl-2-[[(9Z,12Z)-1-oxo-9,12-octadecadienyl]oxy]-1-propanaminiumHMDB
9,12-OctadecadienoylcarnitineHMDB
9,12-OctadecadienylcarnitineHMDB
alpha-LinoleoylcarnitineHMDB
Linoleic acid carnitineHMDB
Linoleoyl carnitineHMDB
Chemical FormulaC25H45NO4
Average Molecular Weight423.6291
Monoisotopic Molecular Weight423.334858933
IUPAC Name(3R)-3-[(9Z,12Z)-octadeca-9,12-dienoyloxy]-4-(trimethylazaniumyl)butanoate
Traditional Namelinoleoylcarnitine
CAS Registry Number36816-10-1
SMILES
CCCCC\C=C/C\C=C/CCCCCCCC(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C
InChI Identifier
InChI=1S/C25H45NO4/c1-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-25(29)30-23(21-24(27)28)22-26(2,3)4/h9-10,12-13,23H,5-8,11,14-22H2,1-4H3/b10-9-,13-12-/t23-/m1/s1
InChI KeyMJLXQSQYKZWZCB-DQFWFXSYSA-N
Chemical Taxonomy
DescriptionThis compound 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
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

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 Solubility8.6e-06 g/LALOGPS
logP1.46ALOGPS
logP2.2ChemAxon
logS-7.7ALOGPS
pKa (Strongest Acidic)4.22ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 ŲChemAxon
Rotatable Bond Count20ChemAxon
Refractivity148.51 m³·mol⁻¹ChemAxon
Polarizability51.3 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 (Non-derivatized) - 70eV, Positivesplash10-00di-9230000000-8e303f952de7b09a4059View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-074l-0953800000-c042c48279e03c6942ddView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0006-1920000000-23f60e51d007ae34b3c8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-4930000000-838683bf758f3bdc0bfdView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00di-1031900000-9dae0fd9ea2fd4d9a94aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-03k9-1179400000-b3c183368ca837a1abb3View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-06vu-5090000000-1b5b91637ffacc86b31fView in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.06 +/- 0.03 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.040-0.080 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified0.05 +/- 0.02 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.05(0.02) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.51 uMInfant (0-1 year old)MaleNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.36 +/- 0.23 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.14 +/- 0.09 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.36 +/- 0.17 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.1 +/- 0.07 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.10 (0.04-0.30) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.0007 (0.0003-0.002) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.042 +/- 0.011 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.07(0.03) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified0.045 +/- 0.010 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.06(0.02) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected and Quantified0.0317 (0.0179) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified0.0208 (0.0151) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified0.0498 (0.0156) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified2.35 uMInfant (0-1 year old)MaleGlutaric aciduria II details
Associated Disorders and Diseases
Disease References
Pregnancy
  1. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Obesity
  1. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Glutaric aciduria II
  1. Domizio S, Romanelli A, Brindisino P, Puglielli C, Conte E, Domizio R, Sgarrella MC, Sabatino G: Glutaric aciduria type II: a case report. Int J Immunopathol Pharmacol. 2005 Oct-Dec;18(4):805-8. [PubMed:16388731 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB023928
KNApSAcK IDNot Available
Chemspider ID4952667
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG ID2614398
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound6450015
PDB IDNot Available
ChEBI ID84098
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Reuter SE, Evans AM, Faull RJ, Chace DH, Fornasini G: Impact of haemodialysis on individual endogenous plasma acylcarnitine concentrations in end-stage renal disease. Ann Clin Biochem. 2005 Sep;42(Pt 5):387-93. [PubMed:16168195 ]
  2. Gempel K, Kiechl S, Hofmann S, Lochmuller H, Kiechl-Kohlendorfer U, Willeit J, Sperl W, Rettinger A, Bieger I, Pongratz D, Gerbitz KD, Bauer MF: Screening for carnitine palmitoyltransferase II deficiency by tandem mass spectrometry. J Inherit Metab Dis. 2002 Feb;25(1):17-27. [PubMed:11999976 ]
  3. Shen JJ, Matern D, Millington DS, Hillman S, Feezor MD, Bennett MJ, Qumsiyeh M, Kahler SG, Chen YT, Van Hove JL: Acylcarnitines in fibroblasts of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders. J Inherit Metab Dis. 2000 Feb;23(1):27-44. [PubMed:10682306 ]
  4. Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Exil V, Duran M, Wijburg FA, Wanders RJ, Strauss AW: Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress. Eur J Clin Invest. 2004 Mar;34(3):191-6. [PubMed:15025677 ]