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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:25:06 UTC
HMDB IDHMDB00222
Secondary Accession Numbers
  • HMDB00846
Metabolite Identification
Common NameL-Palmitoylcarnitine
DescriptionL-Palmitoylcarnitine is 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. L-palmitoylcarnitine, due to its amphipatic character is, like detergents, a surface-active molecule and by changing the membrane fluidity and surface charge can change activity of several enzymes and transporters localized in the membrane. L-palmitoylcarnitine has been also reported to change the activity of certain proteins. On the contrary to carnitine, palmitoylcarnitine was shown to stimulate the activity of caspases 3, 7 and 8 and the level of this long-chain acylcarnitine increased during apoptosis. Palmitoylcarnitine was also reported to diminish completely binding of phorbol esters, the protein kinase C activators and to decrease the autophosphorylation of the enzyme. Apart from these isoform nonspecific phenomena, palmitoylcarnitine was also shown to be responsible for retardation in cytoplasm of protein kinase C isoforms β and δ and, in the case of the latter one, to decrease its interaction with GAP-43. Some of the physico-chemical 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 contribute to myocardial damage through alterations in membrane molecular dynamics , one mechanism through which could play an important role in ischemic injury. Palmitoylcarnitine is characteristically elevated in carnitine palmitoyltransferase II deficiency, late-onset (OMIM 255110 ). (PMID 2540838 , 15363641 , 8706815 ).
Structure
Thumb
Synonyms
  1. (+)-palmitoylcarnitine
  2. (3S)-3-hexadecanoyloxy-4-(trimethylammonio)butanoate
  3. (3S)-3-hexadecanoyloxy-4-(trimethylammonio)butanoic acid
  4. (3S)-3-palmitoyloxy-4-(trimethylammonio)butanoate
  5. (3S)-3-palmitoyloxy-4-(trimethylammonio)butanoic acid
  6. 3-Carboxy-N,N,N-trimethyl-2-[(1-oxohexadecyl)oxy]-1-Propanaminium
  7. D-Palmitylcarnitine
  8. Hexadecanoyl-L-carnitine
  9. Hexadecenoyl carnitine
  10. L(-)-Palmitylcarnitine
  11. L-Carnitine palmitoyl ester
  12. L-Palmitoyl-L-carnitine
  13. Palmitoyl D-carnitine
  14. Palmitoyl-(-)-carnitine
  15. Palmitoyl-L-carnitine
  16. Palmityl-L-carnitine
Chemical FormulaC23H45NO4
Average Molecular Weight399.6077
Monoisotopic Molecular Weight399.334858933
IUPAC Name(3R)-3-(hexadecanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional IUPAC 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/t21-/m1/s1
InChI KeyXOMRRQXKHMYMOC-OAQYLSRUSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassFatty Acid Esters
Sub ClassAcyl Carnitines
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Fatty acyl carnitines(KEGG)
  • Fatty acyl carnitines(Lipidmaps)
  • Organic Compounds
Substituents
  • Carboxylic Acid Ester
  • Carboxylic Acid Salt
  • Carnitine
  • Choline
  • Dicarboxylic Acid Derivative
  • Quaternary Ammonium Salt
Direct ParentAcyl Carnitines
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Fatty acid metabolism
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
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 solubility1.200E-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 area66.43ChemAxon
rotatable bond count20ChemAxon
refractivity137.08ChemAxon
polarizability50.41ChemAxon
Spectra
SpectraMS/MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Fatty acid MetabolismSMP00051map00071
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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
UrineDetected and Quantified0.0022 (0.0020-0.0030) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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
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
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
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021910
KNApSAcK IDNot Available
Chemspider ID10128117
KEGG Compound IDC02990
BioCyc IDCPD-419
BiGG ID40966
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00222
Metagene LinkHMDB00222
METLIN ID5231
PubChem Compound11953816
PDB IDNot Available
ChEBI ID17490
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

Enzymes

General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT1B
Uniprot ID:
Q92523
Molecular weight:
83890.705
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-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
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-Palmitoylcarnitinedetails
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT2
Uniprot ID:
P23786
Molecular weight:
73776.335
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-Palmitoylcarnitinedetails
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT1C
Uniprot ID:
Q8TCG5
Molecular weight:
89712.575
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-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