You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version3.6
Creation Date2006-05-22 14:17:32 UTC
Update Date2014-10-29 17:15:02 UTC
HMDB IDHMDB02013
Secondary Accession NumbersNone
Metabolite Identification
Common NameButyrylcarnitine
DescriptionButyrylcarnitine is elevated in patients with acyl-coa dehydrogenase, short-chain (SCAD) deficiencyin; 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
  1. (3-carboxy-2-hydroxypropyl)trimethyl-L-butyrate
  2. (R)-3-carboxy-N,N,N-trimethyl-2-(1-oxobutoxy)-1-Propanaminium
  3. Butyryl-L-carnitine
  4. Ester with (3-carboxy-2-hydroxypropyl)trimethylammonium L-Butyric acid
  5. Ester with L-(3-carboxy-2-hydroxypropyl)trimethylammonium Butyric acid
  6. L-Carnitine butyryl ester
  7. N-Butyryl-L(-)-carnitine
  8. O-Butanoylcarnitine
Chemical FormulaC11H21NO4
Average Molecular Weight231.2887
Monoisotopic Molecular Weight231.147058165
IUPAC Name3-(butanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional Name3-(butanoyloxy)-4-(trimethylaminio)butanoate
CAS Registry Number25576-40-3
SMILES
CCCC(=O)OC(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
InChI KeyQWYFHHGCZUCMBN-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassFatty Acid Esters
Sub ClassAcyl Carnitines
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Fatty acyl carnitines(KEGG)
  • 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 Solubility0.052ALOGPS
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 Å2ChemAxon
Rotatable Bond Count8ChemAxon
Refractivity81.86 m3·mol-1ChemAxon
Polarizability24.75 Å3ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Fibroblasts
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.26 +/- 0.16 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified0.267 +/- 0.013 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
SalivaDetected and Quantified0.073 +/- 0.044 uMAdult (>18 years old)BothNormal
    • Dame, ZT. et al. ...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.76 (0.12-1.81) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.152 +/- 0.011 uMAdult (>18 years old)BothCeliac disease details
BloodDetected and Quantified0.005 (0.0-0.009) 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
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
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022795
KNApSAcK IDNot Available
Chemspider ID388877
KEGG Compound IDC02862
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB02013
Metagene LinkHMDB02013
METLIN ID6436
PubChem Compound439829
PDB IDNot Available
ChEBI IDNot Available
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. OMMBID - The Metabolic and Molecular Bases of Inherited Disease