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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2022-03-07 02:49:00 UTC
HMDB IDHMDB0000222
Secondary Accession Numbers
  • HMDB0000846
  • HMDB00222
  • HMDB00846
Metabolite Identification
Common NamePalmitoylcarnitine
DescriptionL-Palmitoylcarnitine or hexadecanoylcarnitine is an acylcarnitine. It is technically a long-chain acyl fatty acid derivative ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, palmitic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called palmitoyl-CoA. This reaction is facilitated by the Long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, palmitoyl-CoA reacts with L-carnitine to form palmitoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the palmitoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, palmitoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form palmitoyl-CoA and L-carnitine. Palmitoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing palmitoylcarnitine from forming and thereby preventing it from being transported into the mitochondria. L-Palmitoylcarnitine has been also reported to change the activity of certain proteins and to stimulate the activity of caspases 3, 7, and 8. Interestingly, the level of this long-chain acylcarnitine has been shown to increase during apoptosis. Palmitoylcarnitine has also been reported to diminish the binding of phorbol esters (protein kinase C activators) and the autophosphorylation of the enzyme. Some of the physicochemical properties of palmitoylcarnitine may help to explain the need for coenzyme A-carnitine-coenzyme A acyl exchange during mitochondrial fatty acid import. The amphiphilic character of palmitoylcarnitine may also explain its proposed involvement in the pathogenesis of myocardial ischemia. L-Palmitoylcarnitine accumulates in ischemic myocardium and potentially contributes to myocardial damage through alterations in membrane molecular dynamics. This is a mechanism through which could play an important role in ischemic injury (PMID: 2540838 , 15363641 , 8706815 ). Palmitoylcarnitine is characteristically elevated in late-onset carnitine palmitoyltransferase II deficiency (OMIM: 255110 ).
Structure
Data?1614283473
Synonyms
ValueSource
(3R)-3-(Hexadecanoyloxy)-4-(trimethylazaniumyl)butanoateChEBI
(3R)-3-Palmitoyloxy-4-(trimethylammonio)butanoateChEBI
Hexadecanoyl-L-carnitineChEBI
HexadecanoylcarnitineChEBI
Hexadecenoyl carnitineChEBI
L-Carnitine palmitoyl esterChEBI
O-Hexadecanoyl-(R)-carnitineChEBI
O-Hexadecanoyl-R-carnitineChEBI
Palmitoyl-L-carnitineChEBI
PalmitoylcarnitineChEBI
(3R)-3-(Hexadecanoyloxy)-4-(trimethylazaniumyl)butanoic acidGenerator
(3R)-3-Palmitoyloxy-4-(trimethylammonio)butanoic acidGenerator
(+)-PalmitoylcarnitineHMDB
3-Carboxy-N,N,N-trimethyl-2-[(1-oxohexadecyl)oxy]-1-propanaminiumHMDB
L(-)-PalmitylcarnitineHMDB
L-Palmitoyl-L-carnitineHMDB
Palmitoyl-(-)-carnitineHMDB
Palmityl-L-carnitineHMDB
PalmitylcarnitineHMDB
(2R)-PalmitoylcarnitineHMDB
C16 CarnitineHMDB
L-PalmitoylcarnitineHMDB
Chemical FormulaC23H46NO4
Average Molecular Weight400.623
Monoisotopic Molecular Weight400.342135385
IUPAC Name(3R)-3-(hexadecanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional Namepalmitoylcarnitine
CAS Registry Number2364-67-2
SMILES
CCCCCCCCCCCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C23H45NO4/c1-5-6-7-8-9-10-11-12-13-14-15-16-17-18-23(27)28-21(19-22(25)26)20-24(2,3)4/h21H,5-20H2,1-4H3/p+1/t21-/m1/s1
InChI KeyXOMRRQXKHMYMOC-OAQYLSRUSA-O
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
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
PropertyValueSource
Water Solubility1.2e-05 g/LALOGPS
logP1.77ALOGPS
logP2.03ChemAxon
logS-7.6ALOGPS
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
Refractivity137.08 m³·mol⁻¹ChemAxon
Polarizability50.35 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DeepCCS[M+H]+214.76730932474
DeepCCS[M-H]-210.74830932474
DeepCCS[M-2H]-247.29130932474
DeepCCS[M+Na]+223.58230932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
PalmitoylcarnitineCCCCCCCCCCCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C3731.4Standard polar33892256
PalmitoylcarnitineCCCCCCCCCCCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C2561.0Standard non polar33892256
PalmitoylcarnitineCCCCCCCCCCCCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C2785.1Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Palmitoylcarnitine,1TMS,isomer #1CCCCCCCCCCCCCCCC(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C2795.6Semi standard non polar33892256
Palmitoylcarnitine,1TBDMS,isomer #1CCCCCCCCCCCCCCCC(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C3032.8Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Palmitoylcarnitine GC-MS (Non-derivatized) - 70eV, Positivesplash10-00di-9220000000-890219021b84f68945402016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Palmitoylcarnitine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Palmitoylcarnitine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Palmitoylcarnitine GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Palmitoylcarnitine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-0udi-1000900000-109f35c9819eb5eed7b72012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-000i-9000000000-9d6bb14eddb4cfa8341a2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-000i-9000000000-87b1d0debe4e0cb7bd312012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Linear Ion Trap , negative-QTOFsplash10-014i-0139500000-21c36c851939b94d45232017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Linear Ion Trap , negative-QTOFsplash10-014i-0029600000-69ebe292c2ce42c8d8102017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Linear Ion Trap , positive-QTOFsplash10-0f6x-0139600000-197944fd1aa4ac44bdd22017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Linear Ion Trap , positive-QTOFsplash10-0uxr-0000613900-006582b143e5906e51cc2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Linear Ion Trap , positive-QTOFsplash10-0uxr-0000614900-14a5d773f652b71671fb2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Palmitoylcarnitine Linear Ion Trap , positive-QTOFsplash10-0f6x-0139600000-b0528be9c13e412551af2017-09-14HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Palmitoylcarnitine 10V, Positive-QTOFsplash10-0udi-0000900000-b54b0a7f2164f15f15f22021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Palmitoylcarnitine 20V, Positive-QTOFsplash10-0f79-9000500000-d7434e08a3e0942fa2e92021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Palmitoylcarnitine 40V, Positive-QTOFsplash10-000i-9000000000-e9262cbaff8cb4ad0ba62021-09-24Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, H2O, predicted)2022-08-20Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, CD3OD, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.153 (0.073-0.227) uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified0.113 +/- 0.006 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.050-0.180 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.15 +/- 0.17 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified0.06 +/- 0.04 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified0.11 +/- 0.03 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.12(0.03) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.050-0.180 uMAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.36 +/- 0.27 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.22 +/- 0.07 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified0.034 +/- 0.012 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.01-0.06 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.01(0.01-0.07) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified0.01(0.01-0.03) umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified0.0022 (0.0020-0.0030) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.006 +/- 0.0018 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified0.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.909 (1.012-2.233) uMAdult (>18 years old)BothVery long-chain acyl-CoA dehydrogenase deficiency (vLCAD) details
BloodDetected and Quantified0.097 +/- 0.006 uMAdult (>18 years old)BothCeliac disease details
BloodDetected and Quantified0.13 +/- 0.09 uMChildren (1-13 years old)Both
Acetaminophen overdose
details
BloodDetected and Quantified0.046 (0.0375) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified0.14(0.04) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified0.096 +/- 0.032 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.101 +/- 0.026 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Bothliver cirrhosis details
UrineDetected and Quantified0.0046 +/- 0.0018 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified0.0064 +/- 0.0047 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Analysis of 30 no...
details
Associated Disorders and Diseases
Disease References
Celiac disease
  1. Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed:16425363 ]
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.
Cirrhosis
  1. Huang HJ, Zhang AY, Cao HC, Lu HF, Wang BH, Xie Q, Xu W, Li LJ: Metabolomic analyses of faeces reveals malabsorption in cirrhotic patients. Dig Liver Dis. 2013 Aug;45(8):677-82. doi: 10.1016/j.dld.2013.01.001. Epub 2013 Feb 4. [PubMed:23384618 ]
Colorectal cancer
  1. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021910
KNApSAcK IDNot Available
Chemspider ID10128117
KEGG Compound IDC02990
BioCyc IDCPD-419
BiGG ID40966
Wikipedia LinkPalmitoylcarnitine
METLIN ID5231
PubChem Compound11953816
PDB IDNot Available
ChEBI ID17490
Food Biomarker OntologyNot Available
VMH IDPMTCRN
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceNorum, Kaare R. Palmityl coenzyme A-carnitine palmityltransferase. Purification from calf-liver mitochondria and some properties of the enzyme. Biochimica et Biophysica Acta, Specialized Section on Enzymological Subjects (1964), 89(1), 95-108.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Kamimori H, Hamashima Y, Konishi M: Determination of carnitine and saturated-acyl group carnitines in human urine by high-performance liquid chromatography with fluorescence detection. Anal Biochem. 1994 May 1;218(2):417-24. [PubMed:8074302 ]
  2. Mueller P, Schulze A, Schindler I, Ethofer T, Buehrdel P, Ceglarek U: Validation of an ESI-MS/MS screening method for acylcarnitine profiling in urine specimens of neonates, children, adolescents and adults. Clin Chim Acta. 2003 Jan;327(1-2):47-57. [PubMed:12482618 ]
  3. Moder M, Kiessling A, Loster H, Bruggemann L: The pattern of urinary acylcarnitines determined by electrospray mass spectrometry: a new tool in the diagnosis of diabetes mellitus. Anal Bioanal Chem. 2003 Jan;375(2):200-10. Epub 2003 Jan 4. [PubMed:12560963 ]
  4. Wasant P, Matsumoto I, Naylor E, Liammongkolkul S: Mitochondrial fatty acid oxidation disorders in Thai infants: a report of 3 cases. J Med Assoc Thai. 2002 Aug;85 Suppl 2:S710-9. [PubMed:12403251 ]
  5. Young SP, Matern D, Gregersen N, Stevens RD, Bali D, Liu HM, Koeberl DD, Millington DS: A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency. Clin Chim Acta. 2003 Nov;337(1-2):103-13. [PubMed:14568186 ]
  6. Poorthuis BJ, Jille-Vlckova T, Onkenhout W: Determination of acylcarnitines in urine of patients with inborn errors of metabolism using high-performance liquid chromatography after derivatization with 4'-bromophenacylbromide. Clin Chim Acta. 1993 Jul 16;216(1-2):53-61. [PubMed:8222273 ]
  7. Bhuiyan AK, Jackson S, Turnbull DM, Aynsley-Green A, Leonard JV, Bartlett K: The measurement of carnitine and acyl-carnitines: application to the investigation of patients with suspected inherited disorders of mitochondrial fatty acid oxidation. Clin Chim Acta. 1992 May 15;207(3):185-204. [PubMed:1327583 ]
  8. Nalecz KA, Miecz D, Berezowski V, Cecchelli R: Carnitine: transport and physiological functions in the brain. Mol Aspects Med. 2004 Oct-Dec;25(5-6):551-67. [PubMed:15363641 ]
  9. Watanabe H, Kobayashi A, Hayashi H, Yamazaki N: Effects of long-chain acyl carnitine on membrane fluidity of human erythrocytes. Biochim Biophys Acta. 1989 Apr 28;980(3):315-8. [PubMed:2540838 ]
  10. Goni FM, Requero MA, Alonso A: Palmitoylcarnitine, a surface-active metabolite. FEBS Lett. 1996 Jul 15;390(1):1-5. [PubMed:8706815 ]
  11. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Enzymes

General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT1B
Uniprot ID:
Q92523
Molecular weight:
83890.705
Reactions
Palmityl-CoA + L-Carnitine → Coenzyme A + Palmitoylcarnitinedetails
General function:
Involved in acyltransferase activity
Specific function:
Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism.
Gene Name:
CPT1A
Uniprot ID:
P50416
Molecular weight:
86238.415
Reactions
Palmityl-CoA + L-Carnitine → Coenzyme A + Palmitoylcarnitinedetails
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT2
Uniprot ID:
P23786
Molecular weight:
73776.335
Reactions
Palmityl-CoA + L-Carnitine → Coenzyme A + Palmitoylcarnitinedetails
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT1C
Uniprot ID:
Q8TCG5
Molecular weight:
89712.575
Reactions
Palmityl-CoA + L-Carnitine → Coenzyme A + Palmitoylcarnitinedetails
General function:
Involved in catalytic activity
Specific function:
Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses palmitoleate, oleate and linoleate.
Gene Name:
ACSL1
Uniprot ID:
P33121
Molecular weight:
77942.685