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Showing metabocard for 3-Hydroxybutyrylcarnitine (HMDB0013127)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesXML
Record Information
Version4.0
StatusDetected and Quantified
Creation Date2009-11-02 23:11:25 UTC
Update Date2020-11-09 23:18:40 UTC
HMDB IDHMDB0013127
Secondary Accession Numbers
  • HMDB13127
Metabolite Identification
Common Name3-Hydroxybutyrylcarnitine
Description3-Hydroxybutyrylcarnitine, also known as hydroxybutyrylcarnitine, 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. 3-Hydroxybutyrylcarnitine is considered to be a slightly soluble (in water) and a weak acidic compound. 3-Hydroxybutyrylcarnitine is a fatty ester lipid molecule. 3-Hydroxybutyrylcarnitine can be found in blood. Within a cell, 3-hydroxybutyrylcarnitine is primarily located in the extracellular space and near the membrane.
Structure
Data?1582753096
MOLSDFPDBSMILESInChI
Synonyms
ValueSource
(3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acidGenerator
(3S)-HydroxybutyrylcarnitineHMDB
(S)-3-HydroxybutyrylcarnitineHMDB
3-HydroxybutyrylcarnitineHMDB
HydroxybutyrylcarnitineHMDB
Chemical FormulaC11H21NO5
Average Molecular Weight247.291
Monoisotopic Molecular Weight247.14197278
IUPAC Name(3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Name(3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylammonio)butanoate
CAS Registry Number1469900-92-2
SMILES
C[C@@H](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+/m1/s1
InChI KeyUEFRDQSMQXDWTO-BDAKNGLRSA-N
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
  • Beta-hydroxy acid
  • Hydroxy acid
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Secondary alcohol
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organic salt
  • Organic zwitterion
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Alcohol
  • 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
logP-2.3ALOGPS
logP-4.6ChemAxon
logS-2.4ALOGPS
pKa (Strongest Acidic)4.14ChemAxon
pKa (Strongest Basic)-2.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area86.66 ŲChemAxon
Rotatable Bond Count8ChemAxon
Refractivity83.46 m³·mol⁻¹ChemAxon
Polarizability25.51 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
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 QuantifiedNot QuantifiedAdult (>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
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal 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 QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
Associated Disorders and Diseases
Disease References
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 ]
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 ]
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 ]
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 ]
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 ]
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
FooDB IDNot Available
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem CompoundNot Available
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00029837
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. 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 ]
  6. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.