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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-05-22 14:17:32 UTC
Update Date2020-02-26 21:23:43 UTC
HMDB IDHMDB0002014
Secondary Accession Numbers
  • HMDB02014
Metabolite Identification
Common Namecis-5-Tetradecenoylcarnitine
DescriptionTetradecenoylcarnitine (C14:1) is the most characteristic metabolic marker of very long chain acyl-dehydrogenase (VLCAD) deficiency (PMID: 11433098 ). beta-Oxidation of long-chain fatty acids provides the major source of energy in the heart. Defects in enzymes of the beta-oxidation pathway cause sudden, unexplained death in childhood, acute hepatic encephalopathy or liver failure, skeletal myopathy, and cardiomyopathy (PMID: 7479827 ).
Structure
Data?1582752223
Synonyms
ValueSource
3-[(5Z)-Tetradec-5-enoyloxy]-4-(trimethylammonio)butanoateChEBI
3-[(5Z)-Tetradec-5-enoyloxy]-4-(trimethylammonio)butanoic acidGenerator
cis-5-TetradecenoylcarnitineChEBI
Chemical FormulaC21H39NO4
Average Molecular Weight369.5387
Monoisotopic Molecular Weight369.287908741
IUPAC Name3-[(5Z)-tetradec-5-enoyloxy]-4-(trimethylazaniumyl)butanoate
Traditional Namecis-5-tetradecenoylcarnitine
CAS Registry Number835598-21-5
SMILES
CCCCCCCC\C=C/CCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C21H39NO4/c1-5-6-7-8-9-10-11-12-13-14-15-16-21(25)26-19(17-20(23)24)18-22(2,3)4/h12-13,19H,5-11,14-18H2,1-4H3/b13-12-
InChI KeyNNCBVXBBLABOCB-SEYXRHQNSA-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
  • 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
Physiological effect

Health effect:

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 Solubility1.7e-05 g/LALOGPS
logP0.48ALOGPS
logP0.78ChemAxon
logS-7.4ALOGPS
pKa (Strongest Acidic)4.24ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 ŲChemAxon
Rotatable Bond Count17ChemAxon
Refractivity128.99 m³·mol⁻¹ChemAxon
Polarizability44.6 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00di-9210000000-d55933555203a81af3b0Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0fkc-0729000000-b7724178fd672abeda7cSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0006-1900000000-a2f60fad8c99a570c7b0Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-02fx-4900000000-6ae1e898595b8e8b61f5Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-0029000000-a5108e2a8a5c949c7942Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0aor-1289000000-351e00d1ad0b6a3015e9Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a6u-7590000000-8eeb0dac52aa2c1d6246Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.06 +/- 0.03 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.020-0.240 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified0.03 (0.03-0.04) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified0.020-0.240 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.08 (0.06-0.11) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified<1 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified0.032 (0.005-0.097) uMChildren (1-13 years old)BothNormal details
UrineDetected and Quantified0.0038 +/- 0.0015 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.1014 (0.0757) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified0.122 +/- 0.042 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.131 +/- 0.048 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified5.223 (2.658-7.891) uMAdult (>18 years old)BothVery long-chain acyl-CoA dehydrogenase deficiency (vLCAD) details
UrineDetected and Quantified0.0039 +/- 0.0013 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.0089 +/- 0.0114 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
Very Long Chain Acyl-CoA Dehydrogenase Deficiency
  1. Costa CG, Struys EA, Bootsma A, ten Brink HJ, Dorland L, Tavares de Almeida I, Duran M, Jakobs C: Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. J Lipid Res. 1997 Jan;38(1):173-82. [PubMed:9034211 ]
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.
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Associated OMIM IDs
  • 201475 (Very Long Chain Acyl-CoA Dehydrogenase Deficiency)
  • 601665 (Obesity)
  • 610247 (Eosinophilic esophagitis)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022796
KNApSAcK IDNot Available
Chemspider ID17216151
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6437
PubChem Compound22833575
PDB IDNot Available
ChEBI ID73060
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF: Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood. Proc Natl Acad Sci U S A. 1995 Nov 7;92(23):10496-500. [PubMed:7479827 ]
  2. Shigematsu Y, Hirano S, Hata I, Tanaka Y, Sudo M, Tajima T, Sakura N, Yamaguchi S, Takayanagi M: Selective screening for fatty acid oxidation disorders by tandem mass spectrometry: difficulties in practical discrimination. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):63-72. [PubMed:12828998 ]
  3. Wood JC, Magera MJ, Rinaldo P, Seashore MR, Strauss AW, Friedman A: Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card. Pediatrics. 2001 Jul;108(1):E19. [PubMed:11433098 ]
  4. Archie JP Jr: Splenic artery stump back pressure. Am Surg. 1992 Aug;58(8):504-5. [PubMed:1642390 ]