Human Metabolome Database Version 3.5

Showing metabocard for L-Palmitoylcarnitine (HMDB00222)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:25:06 -0600
HMDB ID HMDB00222
Secondary Accession Numbers
  • HMDB00846
Metabolite Identification
Common Name L-Palmitoylcarnitine
Description L-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 Link_out). (PMID 2540838 Link_out, 15363641 Link_out, 8706815 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
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 Formula C23H45NO4
Average Molecular Weight 399.6077
Monoisotopic Molecular Weight 399.334858933
IUPAC Name (3R)-3-(hexadecanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional IUPAC Name palmitoylcarnitine
CAS Registry Number 2364-67-2
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 Key XOMRRQXKHMYMOC-OAQYLSRUSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Lipids
Class Fatty Acid Esters
Sub Class Acyl 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 Parent Acyl Carnitines
Ontology
Status Detected 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
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 1.200E-05 g/L ALOGPS
LogP 1.77 ALOGPS
LogP 2.03 ChemAxon
LogS -7.58 ALOGPS
pKa (strongest acidic) 4.22 ChemAxon
pKa (strongest basic) -7.1 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 0 ChemAxon
Polar Surface Area 66.43 A2 ChemAxon
Rotatable Bond Count 20 ChemAxon
Refractivity 137.08 ChemAxon
Polarizability 50.41 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Urine
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Fatty acid Metabolism SMP00051 map00071 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
0.153 (0.073-0.227) uM Children (1-13 year old) Both Normal
Blood Detected and Quantified
0.113 +/- 0.006 uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.0022 (0.0020-0.0030) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
0.5 umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 1.909 (1.012-2.233) uM Adult (>18 years old) Both Very long-chain acyl-CoA dehydrogenase deficiency (vLCAD)
Blood Detected and Quantified 0.097 +/- 0.006 uM Adult (>18 years old) Both Celiac disease
Associated Disorders and Diseases
Disease References
Celiac disease
  • 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 Link_out
      Very Long Chain Acyl-CoA Dehydrogenase Deficiency
      • 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 Link_out
          Associated OMIM IDs
          DrugBank ID Not Available
          DrugBank Metabolite ID Not Available
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB021910
          KNApSAcK ID Not Available
          Chemspider ID 10128117 Link_out
          KEGG Compound ID C02990 Link_out
          BioCyc ID CPD-419 Link_out
          BiGG ID 40966 Link_out
          Wikipedia Link Not Available
          NuGOwiki Link HMDB00222 Link_out
          Metagene Link HMDB00222 Link_out
          METLIN ID 5231 Link_out
          PubChem Compound 11953816 Link_out
          PDB ID Not Available
          ChEBI ID 17490 Link_out
          References
          Synthesis Reference Norum, 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. 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 Link_out
          2. 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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out
          8. 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 Link_out
          9. 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 Link_out
          10. Goni FM, Requero MA, Alonso A: Palmitoylcarnitine, a surface-active metabolite. FEBS Lett. 1996 Jul 15;390(1):1-5. Pubmed: 8706815 Link_out

          Enzymes
          Name: Carnitine O-palmitoyltransferase 1, muscle isoform
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT1B
          Uniprot ID: Q92523 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 1, liver isoform
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT1A
          Uniprot ID: P50416 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 2, mitochondrial
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT2
          Uniprot ID: P23786 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 1, brain isoform
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT1C
          Uniprot ID: Q8TCG5 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Long-chain-fatty-acid--CoA ligase 1
          Reactions: Not Available
          Gene Name: ACSL1
          Uniprot ID: P33121 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA