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Record Information
Version3.6
Creation Date2006-05-22 14:17:50 UTC
Update Date2016-02-11 01:05:36 UTC
HMDB IDHMDB02366
Secondary Accession NumbersNone
Metabolite Identification
Common NameTiglylcarnitine
DescriptionTiglylcarnitine is detected in the urinary organic acid and blood spot acylcarnitine profiles in patients with mitochondrial acetoacetyl-CoA thiolase (T2) deficiency -- an inborn error of metabolism affecting isoleucine and ketone bodies in the catabolic process. (PubMed ID 14518824 ).
Structure
Thumb
Synonyms
ValueSource
3-{[(2E)-2-methylbut-2-enoyl]oxy}-4-(trimethylammonio)butanoateChEBI
Tiglyl-L-carnitineHMDB
Chemical FormulaC12H21NO4
Average Molecular Weight243.2994
Monoisotopic Molecular Weight243.147058165
IUPAC Name3-{[(2E)-2-methylbut-2-enoyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Nametiglylcarnitine
CAS Registry Number64191-86-2
SMILES
C\C=C(/C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C12H21NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h6,10H,7-8H2,1-5H3/b9-6+
InChI KeyInChIKey=WURBQCVBQNMUQT-RMKNXTFCSA-N
Chemical Taxonomy
DescriptionThis compound 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
  • Carnitine
  • Acyl choline
  • Choline
  • Branched fatty acid
  • Dicarboxylic acid or derivatives
  • Alpha,beta-unsaturated carboxylic ester
  • Enoate ester
  • Quaternary ammonium salt
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organic salt
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Organic zwitterion
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
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 Solubility0.023 mg/mLALOGPS
logP-1.9ALOGPS
logP-2.7ChemAxon
logS-4.1ALOGPS
pKa (Strongest Acidic)4.3ChemAxon
pKa (Strongest Basic)-6.9ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 Å2ChemAxon
Rotatable Bond Count7ChemAxon
Refractivity87.32 m3·mol-1ChemAxon
Polarizability25.91 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MSNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.05 +/- 0.01 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.033 +/- 0.003 uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.10 (0.05-0.20) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.034 +/- 0.002 uMAdult (>18 years old)BothCeliac disease details
Associated Disorders and Diseases
Disease References
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. [16425363 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022980
KNApSAcK IDNot Available
Chemspider ID17216200
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB02366
Metagene LinkHMDB02366
METLIN ID6648
PubChem Compound22833596
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Fontaine M, Briand G, Ser N, Armelin I, Rolland MO, Degand P, Vamecq J: Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency. Clin Chim Acta. 1996 Nov 15;255(1):67-83. [8930414 ]
  2. Fukao T, Zhang GX, Sakura N, Kubo T, Yamaga H, Hazama A, Kohno Y, Matsuo N, Kondo M, Yamaguchi S, Shigematsu Y, Kondo N: The mitochondrial acetoacetyl-CoA thiolase (T2) deficiency in Japanese patients: urinary organic acid and blood acylcarnitine profiles under stable conditions have subtle abnormalities in T2-deficient patients with some residual T2 activity. J Inherit Metab Dis. 2003;26(5):423-31. [14518824 ]
  3. Millington DS, Roe CR, Maltby DA: Characterization of new diagnostic acylcarnitines in patients with beta-ketothiolase deficiency and glutaric aciduria type I using mass spectrometry. Biomed Environ Mass Spectrom. 1987 Dec;14(12):711-6. [3435793 ]