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Record Information
Version4.0
Creation Date2006-05-22 14:17:50 UTC
Update Date2017-09-27 08:24:16 UTC
HMDB IDHMDB0002366
Secondary Accession Numbers
  • HMDB02366
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
3-{[(2E)-2-methylbut-2-enoyl]oxy}-4-(trimethylammonio)butanoic acidGenerator
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 KeyWURBQCVBQNMUQT-RMKNXTFCSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentAcyl carnitines
Alternative Parents
Substituents
  • Acyl-carnitine
  • Branched fatty acid
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Alpha,beta-unsaturated carboxylic ester
  • Enoate ester
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic nitrogen compound
  • Organonitrogen compound
  • Organooxygen compound
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

  Health condition:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Cellular process:

    Chemical reaction:

    Biochemical process:

    Biochemical pathway:

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 Solubility0.023 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count7ChemAxon
Refractivity87.32 m³·mol⁻¹ChemAxon
Polarizability25.91 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 (Non-derivatized) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0006-3890000000-658980fc0a49ff84c058View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-002f-4910000000-e52343a18415b605fbdaView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0pir-9100000000-5d51d3482a8530d3a0c2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0006-3190000000-11ea39113a6ecfa2df67View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0544-7940000000-b85e0eef7f13db4025deView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4j-9000000000-2d79ca02f305f46eeb31View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.05 +/- 0.01 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.040-0.080 uMAdult (>18 years old)Both
Normal
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. [PubMed: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
METLIN ID6648
PubChem Compound22833596
PDB IDNot Available
ChEBI ID71179
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. [PubMed: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. [PubMed: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. [PubMed:3435793 ]