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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-05-22 14:17:32 UTC
Update Date2018-05-20 10:11:03 UTC
HMDB IDHMDB0002013
Secondary Accession Numbers
  • HMDB0062510
  • HMDB02013
  • HMDB62510
Metabolite Identification
Common NameButyrylcarnitine
DescriptionButyrylcarnitine, also known as (3R)-3-(butyryloxy)-4-(trimethylammonio)butanoate or L-carnitine butyryl ester, is classified as a member of the acylcarnitines. Acylcarnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Butyrylcarnitine is considered to be practically insoluble (in water) and acidic. Butyrylcarnitine is elevated in patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency, in infants with acute acidosis and generalized muscle weakness, and in middle-aged patients with chronic myopathy localized in muscle (OMIM: 201470 ).
Structure
Thumb
Synonyms
ValueSource
(3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoateChEBI
Butyryl-L-carnitineChEBI
ButyrylcarnitineChEBI
L-Carnitine butyryl esterChEBI
N-Butyryl-L-(-)-carnitinChEBI
O-Butanoyl-(R)-carnitineChEBI
(3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoic acidGenerator
ButylcarnitineHMDB
O-Butanoyl-carnitineHMDB
O-ButanoylcarnitineHMDB
Chemical FormulaC11H21NO4
Average Molecular Weight231.292
Monoisotopic Molecular Weight231.14705816
IUPAC Name(3R)-3-(butanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional NameO-butanoylcarnitine
CAS Registry Number25576-40-3
SMILES
CCCC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C11H21NO4/c1-5-6-11(15)16-9(7-10(13)14)8-12(2,3)4/h9H,5-8H2,1-4H3/t9-/m1/s1
InChI KeyQWYFHHGCZUCMBN-SECBINFHSA-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
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

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 Solubility0.052 g/LALOGPS
logP-2.1ALOGPS
logP-3.3ChemAxon
logS-3.7ALOGPS
pKa (Strongest Acidic)4.27ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 ŲChemAxon
Rotatable Bond Count8ChemAxon
Refractivity81.86 m³·mol⁻¹ChemAxon
Polarizability24.8 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Fibroblasts
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.26 +/- 0.16 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.10-0.45 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified0.267 +/- 0.013 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.10-0.45 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.13 (0.11-0.16) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified0.39 (0.31-0.55) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified<0.12 uMAdolescent (13-18 years old)MaleNormal details
BloodDetected and Quantified0.26(0.1) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.21 +/- 0.09 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.079 (0.023-0.174) uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.024 +/- 0.007 uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected and Quantified0.21 +/- 0.1 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified0.15 +/- 0.06 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified0.073 +/- 0.044 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected and Quantified702.56 +/- 39.76 umol/mmol creatinineAdolescent (13-18 years old)Both
Normal
details
UrineDetected and Quantified0.76 (0.12-1.81) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified2.3594 +/- 1.3471 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.152 +/- 0.011 uMAdult (>18 years old)BothCeliac disease details
BloodDetected and Quantified0.38 uMAdolescent (13-18 years old)MaleGlutaric aciduria II details
BloodDetected and Quantified0.33(0.18) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified0.33(0.14) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected and Quantified0.1378 (0.119) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified0.2169 (0.0736) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified0.005 (0.0-0.009) uMAdult (>18 years old)BothVery long-chain acyl-CoA dehydrogenase deficiency (vLCAD) details
BloodDetected and Quantified0.069 +/- 0.047 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified0.078 +/- 0.035 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
UrineDetected and Quantified565.44 +/- 32.46 umol/mmol creatinineAdolescent (13-18 years old)Both
Obese
details
UrineDetected and Quantified0-8.966 umol/mmol creatinineInfant (0-1 year old)Both
Short chain acyl-CoA dehydrogenase deficiency (SCAD)
details
UrineDetected and Quantified1.368 +/- 1.1114 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.2564 +/- 1.26 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
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 ]
Short Chain Acyl-Coa Dehydrogenase Deficiency
  1. Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [PubMed:7776094 ]
Pregnancy
  1. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
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 ]
Obesity
  1. Cho K, Moon JS, Kang JH, Jang HB, Lee HJ, Park SI, Yu KS, Cho JY: Combined untargeted and targeted metabolomic profiling reveals urinary biomarkers for discriminating obese from normal-weight adolescents. Pediatr Obes. 2017 Apr;12(2):93-101. doi: 10.1111/ijpo.12114. Epub 2016 Feb 22. [PubMed:26910390 ]
  2. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Glutaric aciduria II
  1. Prasad M, Hussain S: Glutaric aciduria type II presenting as myopathy and rhabdomyolysis in a teenager. J Child Neurol. 2015 Jan;30(1):96-9. doi: 10.1177/0883073813516676. Epub 2014 Jan 21. [PubMed:24453145 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022795
KNApSAcK IDNot Available
Chemspider ID184820
KEGG Compound IDC02862
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6436
PubChem Compound213144
PDB IDNot Available
ChEBI ID21949
References
Synthesis ReferenceLozano Pedro; Daz Mirta; de Diego Teresa; Iborra Jose L Ester synthesis from trimethylammonium alcohols in dry organic media catalyzed by immobilized Candida antarctica lipase B. Biotechnology and bioengineering (2003), 82(3), 352-8.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]
  2. Golan R, Soffer Y, Katz S, Weissenberg R, Wasserzug O, Lewin LM: Carnitine and short-chain acylcarnitines in the lumen of the human male reproductive tract. Int J Androl. 1983 Aug;6(4):349-57. [PubMed:6618689 ]
  3. Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [PubMed:7776094 ]
  4. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.
  9. OMMBID - The Metabolic and Molecular Bases of Inherited Disease [Link]