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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2009-11-02 23:11:25 UTC
Update Date2018-05-20 10:11:05 UTC
HMDB IDHMDB0013127
Secondary Accession Numbers
  • HMDB13127
Metabolite Identification
Common NameHydroxybutyrylcarnitine
DescriptionHydroxybutyrylcarnitine, also known as C4OH Carnitine, is classified as a member of the acyl carnitines. Acyl carnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Hydroxybutyrylcarnitine is considered to be a slightly soluble (in water) and a weak acidic compound. Hydroxybutyrylcarnitine is a fatty ester lipid molecule. Hydroxybutyrylcarnitine can be found in blood. Within a cell, Hydroxybutyrylcarnitine is primarily located in the extracellular space and near the membrane.
Structure
Thumb
Synonyms
ValueSource
3-Hydroxy-9(Z)-octadecenoylcarnitineHMDB
Chemical FormulaC11H21NO5
Average Molecular Weight247.2881
Monoisotopic Molecular Weight247.141972787
IUPAC Name(4S)-4-{[(9Z)-3-hydroxyoctadec-9-enoyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Name(4S)-4-{[(9Z)-3-hydroxyoctadec-9-enoyl]oxy}-4-(trimethylammonio)butanoate
CAS Registry NumberNot Available
SMILES
CC(O)CC(=O)O[C@@H](CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C11H21NO5/c1-8(13)5-11(16)17-9(6-10(14)15)7-12(2,3)4/h8-9,13H,5-7H2,1-4H3/t8?,9-/m0/s1
InChI KeyUEFRDQSMQXDWTO-GKAPJAKFSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as beta hydroxy acids and derivatives. These are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassBeta hydroxy acids and derivatives
Direct ParentBeta hydroxy acids and derivatives
Alternative Parents
Substituents
  • Beta-hydroxy acid
  • Fatty acid ester
  • Fatty acyl
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Secondary alcohol
  • Carboxylic acid derivative
  • Carboxylic acid
  • Alcohol
  • Organooxygen compound
  • Organonitrogen compound
  • Organic zwitterion
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Organic nitrogen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
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 Solubility1.1 g/LALOGPS
logP0.73ALOGPS
logP1.66ChemAxon
logS-7.2ALOGPS
pKa (Strongest Acidic)4.06ChemAxon
pKa (Strongest Basic)-2.8ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area86.66 ŲChemAxon
Rotatable Bond Count21ChemAxon
Refractivity148.84 m³·mol⁻¹ChemAxon
Polarizability53.75 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9100000000-4477b542f5098992f775View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.11 +/- 0.01 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.0960 (0.000-0.360) uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified<0.400 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified0.04 +/- 0.02 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.6 +/- 0.36 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.32 +/- 0.2 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.780 (0.920-3.0400) uMInfant (0-1 year old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified0.200 (0.0800-0.500) uMInfant (0-1 year old)Not Specified
Ketosis
details
BloodDetected and Quantified0.180 (0.0600-0.360) uMInfant (0-1 year old)Not Specified
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
details
BloodDetected and Quantified0.940-1.720 uMInfant (0-1 year old)Male3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) details
BloodDetected and Quantified0.660-0.810 uMInfant (0-1 year old)Male3-Hydroxyisobutyryl-coa hydrolase deficiency details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
Associated Disorders and Diseases
Disease References
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 ]
Ketosis
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
  2. Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
3-Hydroxyisobutyryl-coa hydrolase deficiency
  1. Reuter MS, Sass JO, Leis T, Kohler J, Mayr JA, Feichtinger RG, Rauh M, Schanze I, Bahr L, Trollmann R, Uebe S, Ekici AB, Reis A: HIBCH deficiency in a patient with phenotypic characteristics of mitochondrial disorders. Am J Med Genet A. 2014 Dec;164A(12):3162-9. doi: 10.1002/ajmg.a.36766. Epub 2014 Sep 23. [PubMed:25251209 ]
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Associated OMIM IDs
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 250620 (3-Hydroxyisobutyryl-coa hydrolase deficiency)
  • 114500 (Colorectal cancer)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB029400
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound53481699
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  2. Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
  3. Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
  4. Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
  5. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.