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Record Information
StatusDetected and Quantified
Creation Date2006-05-22 14:17:50 UTC
Update Date2021-04-12 19:31:46 UTC
Secondary Accession Numbers
  • HMDB02366
Metabolite Identification
Common NameTiglylcarnitine
DescriptionTiglylcarnitine is an acylcarnitine. More specifically, it is an tiglic acid ester of carnitine. Acylcarnitines were first discovered more than 70 year ago (PMID: 13825279 ). It is believed that there are more than 1000 types of acylcarnitines in the human body. The general role of acylcarnitines is to transport acyl-groups (organic acids and fatty acids) from the cytoplasm into the mitochondria so that they can be broken down to produce energy.  This process is known as beta-oxidation. According to a recent review [Dambrova et al. 2021, Physiological Reviews], acylcarnitines (ACs) can be classified into 9 different categories depending on the type and size of their acyl-group: 1) short-chain ACs; 2) medium-chain ACs; 3) long-chain ACs; 4) very long-chain ACs; 5) hydroxy ACs; 6) branched chain ACs; 7) unsaturated ACs; 8) dicarboxylic ACs and 9) miscellaneous ACs. Short-chain ACs have acyl-groups with two to five carbons (C2-C5), medium-chain ACs have acyl-groups with six to thirteen carbons (C6-C13), long-chain ACs have acyl-groups with fourteen to twenty once carbons (C14-C21) and very long-chain ACs have acyl groups with more than 22 carbons. Tiglylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine Tiglylcarnitine is a member of the most abundant group of carnitines in the body, comprising more than 50% of all acylcarnitines quantified in tissues and biofluids (PMID: 31920980 ). Some short-chain carnitines have been studied as supplements or treatments for a number of diseases, including neurological disorders and inborn errors of metabolism. In particular Tiglylcarnitine is elevated in the blood or plasma of individuals with beta ketothiolase deficiency/acat1 gene mutation (PMID: 27264805 , PMID: 14518824 , PMID: 3435793 ), and ECHS1 deficiency (PMID: 31908952 ). It is also decreased in the blood or plasma of individuals with familial mediterranean fever (PMID: 29900937 ), carcinoma, lewis lung (PMID: 30839735 ), metabolic syndrome, type 2 diabetes mellitus, and cardiovascular diseases (PMID: 24710945 ). Tiglylcarnitine is also 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 (PMID: 14518824 ). Tiglylcarnitine is found to be associated with celiac disease, which is also an inborn error of metabolism. Tiglylcarnitine has been identified in the human placenta (PMID: 32033212 ). Carnitine acetyltransferase (CrAT, EC: is responsible for the synthesis of all short-chain and short branched-chain acylcarnitines (PMID: 23485643 ). The study of acylcarnitines is an active area of research and it is likely that many novel acylcarnitines will be discovered in the coming years. It is also likely that many novel roles in health and disease will be uncovered. An excellent review of the current state of knowledge for acylcarnitines is available at [Dambrova et al. 2021, Physiological Reviews].
Tiglyl carnitineHMDB
Chemical FormulaC12H21NO4
Average Molecular Weight243.303
Monoisotopic Molecular Weight243.14705816
IUPAC Name(3R)-3-{[(2E)-2-methylbut-2-enoyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Name(3R)-3-{[(2E)-2-methylbut-2-enoyl]oxy}-4-(trimethylammonio)butanoate
CAS Registry Number64681-36-3
InChI Identifier
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
  • 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
Physiological effect

Health effect:


Route of exposure:


Biological location:


Naturally occurring process:


Industrial application:

Biological role:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility0.023 g/LALOGPS
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
Polarizability26.09 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Not Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Placenta
Normal Concentrations
BloodDetected and Quantified0.05 +/- 0.01 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.040-0.080 uMAdult (>18 years old)Both
BloodDetected and Quantified0.033 +/- 0.003 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.040-0.080 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified<0.0400 uMNot SpecifiedNot Specified
FecesDetected and Quantified0.24 +/- 0.12 nmol/g wet fecesAdult (>18 years old)Both
FecesDetected and Quantified0.15 +/- 0.08 nmol/g wet fecesAdult (>18 years old)Both
UrineDetected and Quantified0.10 (0.05-0.20) umol/mmol creatinineAdult (>18 years old)Both
UrineDetected and Quantified0.03-0.16 umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • López Hernández Y...
UrineDetected and Quantified0.08 +/- 0.03 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal
    • López Hernández Y...
UrineDetected and Quantified0.07 +/- 0.022 umol/mmol creatinineNewborn (0-30 days old)MaleNormal
    • López Hernández Y...
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
Abnormal Concentrations
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
Phenol Explorer Compound IDNot Available
FooDB IDFDB022980
KNApSAcK IDNot Available
Chemspider ID34999729
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound91825636
PDB IDNot Available
ChEBI ID85520
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB ID
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]
  2. 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 ]
  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 ]
  4. 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 ]
  5. FRITZ IB: Action of carnitine on long chain fatty acid oxidation by liver. Am J Physiol. 1959 Aug;197:297-304. doi: 10.1152/ajplegacy.1959.197.2.297. [PubMed:13825279 ]
  6. Makarova E, Makrecka-Kuka M, Vilks K, Volska K, Sevostjanovs E, Grinberga S, Zarkova-Malkova O, Dambrova M, Liepinsh E: Decreases in Circulating Concentrations of Long-Chain Acylcarnitines and Free Fatty Acids During the Glucose Tolerance Test Represent Tissue-Specific Insulin Sensitivity. Front Endocrinol (Lausanne). 2019 Dec 17;10:870. doi: 10.3389/fendo.2019.00870. eCollection 2019. [PubMed:31920980 ]
  7. Wen P, Chen Z, Wang G, Su Z, Zhang X, Tang G, Cui D, Liu X, Li C: [Analysis of clinical phenotype and ACAT1 gene mutation in a family affected with beta-ketothiolase deficiency]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2016 Jun;33(3):286-91. doi: 10.3760/cma.j.issn.1003-9406.2016.03.002. [PubMed:27264805 ]
  8. Pajares S, Lopez RM, Gort L, Argudo-Ramirez A, Marin JL, Gonzalez de Aledo-Castillo JM, Garcia-Villoria J, Arranz JA, Del Toro M, Tort F, Ugarteburu O, Casellas MD, Fernandez R, Ribes A: An incidental finding in newborn screening leading to the diagnosis of a patient with ECHS1 mutations. Mol Genet Metab Rep. 2020 Jan 2;22:100553. doi: 10.1016/j.ymgmr.2019.100553. eCollection 2020 Mar. [PubMed:31908952 ]
  9. Kiykim E, Aktuglu Zeybek AC, Barut K, Zubarioglu T, Cansever MS, Alsancak S, Kasapcopur O: Screening of Free Carnitine and Acylcarnitine Status in Children With Familial Mediterranean Fever. Arch Rheumatol. 2016 Mar 10;31(2):133-138. doi: 10.5606/ArchRheumatol.2016.5696. eCollection 2016 Jun. [PubMed:29900937 ]
  10. Wu H, Chen Y, Li Z, Liu X: Untargeted metabolomics profiles delineate metabolic alterations in mouse plasma during lung carcinoma development using UPLC-QTOF/MS in MS(E) mode. R Soc Open Sci. 2018 Sep 19;5(9):181143. doi: 10.1098/rsos.181143. eCollection 2018 Sep. [PubMed:30839735 ]
  11. Yu ZR, Ning Y, Yu H, Tang NJ: A HPLC-Q-TOF-MS-based urinary metabolomic approach to identification of potential biomarkers of metabolic syndrome. J Huazhong Univ Sci Technolog Med Sci. 2014 Apr;34(2):276-283. doi: 10.1007/s11596-014-1271-7. Epub 2014 Apr 8. [PubMed:24710945 ]
  12. Violante S, Ijlst L, Ruiter J, Koster J, van Lenthe H, Duran M, de Almeida IT, Wanders RJ, Houten SM, Ventura FV: Substrate specificity of human carnitine acetyltransferase: Implications for fatty acid and branched-chain amino acid metabolism. Biochim Biophys Acta. 2013 Jun;1832(6):773-9. doi: 10.1016/j.bbadis.2013.02.012. Epub 2013 Feb 24. [PubMed:23485643 ]