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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-11-04 20:22:03 UTC
HMDB IDHMDB00705
Secondary Accession NumbersNone
Metabolite Identification
Common NameHexanoylcarnitine
DescriptionHexanoylcarnitine is an acylcarnitine. Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism in the organism which are characterized by the production and excretion of unusual acylcarnitines. A mutation in the gene coding for carnitine-acylcarnitine translocase or the OCTN2 transporter aetiologically causes a carnitine deficiency that results in poor intestinal absorption of dietary L-carnitine, its impaired reabsorption by the kidney and, consequently, in increased urinary loss of L-carnitine. Determination of the qualitative pattern of acylcarnitines can be of diagnostic and therapeutic importance. The betaine structure of carnitine requires special analytical procedures for recording. The ionic nature of L-carnitine causes a high water solubility which decreases with increasing chain length of the ester group in the acylcarnitines. Therefore, the distribution of L-carnitine and acylcarnitines in various organs is defined by their function and their physico-chemical properties as well. High performance liquid chromatography (HPLC) permits screening for free and total carnitine, as well as complete quantitative acylcarnitine determination, including the long-chain acylcarnitine profile. (PMID: 17508264 , Monatshefte fuer Chemie (2005), 136(8), 1279-1291., Int J Mass Spectrom. 1999;188:39-52.). Hexanoylcarnitine is a medium-chain acylcarnitine present in the urine of patients with medium-chain acyl-CoA dehydrogenase deficiency (PubMed ID 1635814 ).
Structure
Thumb
Synonyms
  1. Caproylcarnitine
  2. Hexanoic acid ester with (3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide inner salt
  3. Hexanoyl DL-carnitine
  4. Hexanoyl-D,L-carnitine
  5. Hexanoylcarnitine
Chemical FormulaC13H25NO4
Average Molecular Weight259.3419
Monoisotopic Molecular Weight259.178358293
IUPAC Name3-(hexanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional Name3-(hexanoyloxy)-4-(trimethylaminio)butanoate
CAS Registry Number6418-78-6
SMILES
CCCCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C13H25NO4/c1-5-6-7-8-13(17)18-11(9-12(15)16)10-14(2,3)4/h11H,5-10H2,1-4H3
InChI KeyVVPRQWTYSNDTEA-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassFatty Acid Esters
Sub ClassAcyl Carnitines
Other Descriptors
  • Aliphatic Acyclic Compounds
  • 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
  • Fuel and energy storage
  • Fuel or energy source
  • Lipid catabolism, Fatty acid transport, Energy production
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
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 Solubility0.0043ALOGPS
logP-1.7ALOGPS
logP-2.4ChemAxon
logS-4.9ALOGPS
pKa (Strongest Acidic)4.22ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 Å2ChemAxon
Rotatable Bond Count10ChemAxon
Refractivity91.07 m3·mol-1ChemAxon
Polarizability29.12 Å3ChemAxon
Spectra
SpectraMS/MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Prostate
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.031 (0.015-0.053) uMChildren (1-13 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.001 (0.0-0.004) uMAdult (>18 years old)BothVery long-chain acyl-CoA dehydrogenase deficiency (vLCAD) 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
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022192
KNApSAcK IDNot Available
Chemspider ID4932273
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00705
Metagene LinkHMDB00705
METLIN ID5673
PubChem Compound6426853
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceStrack, Erich; Lorenz, Irmgard. Synthesis of O-acyl derivatives of carnitine. Hoppe-Seyler's Zeitschrift fuer Physiologische Chemie (1966), 343(4-6), 231-9.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Ziadeh R, Hoffman EP, Finegold DN, Hoop RC, Brackett JC, Strauss AW, Naylor EW: Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies. Pediatr Res. 1995 May;37(5):675-8. Pubmed: 7603790
  2. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411
  3. Minkler PE, Hoppel CL: Quantification of free carnitine, individual short- and medium-chain acylcarnitines, and total carnitine in plasma by high-performance liquid chromatography. Anal Biochem. 1993 Aug 1;212(2):510-8. Pubmed: 8214594
  4. Chalmers RA, Roe CR, Stacey TE, Hoppel CL: Urinary excretion of l-carnitine and acylcarnitines by patients with disorders of organic acid metabolism: evidence for secondary insufficiency of l-carnitine. Pediatr Res. 1984 Dec;18(12):1325-8. Pubmed: 6441143
  5. Fontaine M, Briand G, Vallee L, Ricart G, Degand P, Divry P, Vianey-Saban C, Vamecq J: Acylcarnitine removal in a patient with acyl-CoA beta-oxidation deficiency disorder: effect of L-carnitine therapy and starvation. Clin Chim Acta. 1996 Aug 30;252(2):109-22. Pubmed: 8853559
  6. Lee PJ, Harrison EL, Jones MG, Jones S, Leonard JV, Chalmers RA: L-carnitine and exercise tolerance in medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency: a pilot study. J Inherit Metab Dis. 2005;28(2):141-52. Pubmed: 15877203
  7. Tyni T, Pourfarzam M, Turnbull DM: Analysis of mitochondrial fatty acid oxidation intermediates by tandem mass spectrometry from intact mitochondria prepared from homogenates of cultured fibroblasts, skeletal muscle cells, and fresh muscle. Pediatr Res. 2002 Jul;52(1):64-70. Pubmed: 12084849
  8. Caksen H, Tuncer O, Kirimi E, Fryns JP, Uner A, Unal O, Cinal A, Odabas D: Report of two Turkish infants with Norman-Roberts syndrome. Genet Couns. 2004;15(1):9-17. Pubmed: 15083694
  9. Schmidt-Sommerfeld E, Penn D, Rinaldo P, Kossak D, Li BU, Huang ZH, Gage DA: Urinary medium-chain acylcarnitines in medium-chain acyl-CoA dehydrogenase deficiency, medium-chain triglyceride feeding and valproic acid therapy: sensitivity and specificity of the radioisotopic exchange/high performance liquid chromatography method. Pediatr Res. 1992 Jun;31(6):545-51. Pubmed: 1635814
  10. Shigematsu Y, Hata I, Nakai A, Kikawa Y, Sudo M, Tanaka Y, Yamaguchi S, Jakobs C: Prenatal diagnosis of organic acidemias based on amniotic fluid levels of acylcarnitines. Pediatr Res. 1996 Apr;39(4 Pt 1):680-4. Pubmed: 8848345
  11. Schmidt-Sommerfeld E, Penn D, Duran M, Bennett MJ, Santer R, Stanley CA: Detection of inborn errors of fatty acid oxidation from acylcarnitine analysis of plasma and blood spots with the radioisotopic exchange-high-performance liquid chromatographic method. J Pediatr. 1993 May;122(5 Pt 1):708-14. Pubmed: 8496747
  12. 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
  13. Bennett MJ, Coates PM, Hale DE, Millington DS, Pollitt RJ, Rinaldo P, Roe CR, Tanaka K: Analysis of abnormal urinary metabolites in the newborn period in medium-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 1990;13(5):707-15. Pubmed: 2246856
  14. Vallee L, Fontaine M, Nuyts JP, Ricart G, Krivosic I, Divry P, Vianey-Saban C, Lhermitte M, Vamecq J: Stroke, hemiparesis and deficient mitochondrial beta-oxidation. Eur J Pediatr. 1994 Aug;153(8):598-603. Pubmed: 7957409
  15. Schmidt-Sommerfeld E, Penn D, Kerner J, Bieber LL, Rossi TM, Lebenthal E: Quantitation of urinary carnitine esters in a patient with medium-chain acyl-coenzyme A dehydrogenase deficiency: effect of metabolic state and L-carnitine therapy. J Pediatr. 1989 Oct;115(4):577-82. Pubmed: 2795349
  16. Minkler PE, Hoppel CL: Quantification of carnitine and specific acylcarnitines by high-performance liquid chromatography: application to normal human urine and urine from patients with methylmalonic aciduria, isovaleric acidemia or medium-chain acyl-CoA dehydrogenase deficiency. J Chromatogr. 1993 Apr 2;613(2):203-21. Pubmed: 8491807
  17. Pierre G, Macdonald A, Gray G, Hendriksz C, Preece MA, Chakrapani A: Prospective treatment in carnitine-acylcarnitine translocase deficiency. J Inherit Metab Dis. 2007 Oct;30(5):815. Epub 2007 May 12. Pubmed: 17508264