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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2007-05-23 10:45:07 UTC
Update Date2017-10-23 19:04:21 UTC
HMDB IDHMDB0006469
Secondary Accession Numbers
  • 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 ).
Structure
Thumb
SynonymsNot Available
Chemical FormulaC25H45NO4
Average Molecular Weight423.6291
Monoisotopic Molecular Weight423.334858933
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number36816-10-1
SMILESNot Available
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
ClassificationNot classified
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 PropertiesNot Available
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
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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 Quantified0.05(0.02) uMAdult (>18 years old)BothNormal 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
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.07(0.03) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified0.07(0.03) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction 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
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 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 ]