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Record Information
StatusDetected and Quantified
Creation Date2009-11-02 23:32:58 UTC
Update Date2022-10-24 19:44:11 UTC
Secondary Accession Numbers
  • HMDB13128
Metabolite Identification
Common NameValerylcarnitine
DescriptionValerylcarnitine is an acylcarnitine. More specifically, it is an valeric 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 (PMID: 35710135 ), 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. Valerylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine valerylcarnitine 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 valerylcarnitine is elevated in the blood or plasma of individuals with exudative age-related macular degeneration (PMID: 32120889 ), type 2 Diabetes Mellitus (PMID: 31782507 , PMID: 20111019 ), obesity (PMID: 20111019 ), acute cerebral infarction (PMID: 29265114 ), diastolic heart failure (PMID: 26010610 ), systolic heart failure (PMID: 26010610 ). It is also decreased in the blood or plasma of individuals with pregnancy (PMID: 24704061 - in serum of pregnant women with fetus with CHD). Valerylcarnitine can also be found cerebrospinal fluid (CSF), and urine. 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 (PMID: 35710135 ).
Valeryl L-carnitineHMDB
Chemical FormulaC12H23NO4
Average Molecular Weight245.319
Monoisotopic Molecular Weight245.162708225
IUPAC Name(3R)-3-(pentanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional Name(3R)-3-(pentanoyloxy)-4-(trimethylammonio)butanoate
CAS Registry Number40225-14-7
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
  • 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
Physiological effect
Biological locationSource
ProcessNot Available
Physical Properties
Experimental Molecular Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Water Solubility0.016 g/LALOGPS
pKa (Strongest Acidic)4.34ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 ŲChemAxon
Rotatable Bond Count9ChemAxon
Refractivity86.47 m³·mol⁻¹ChemAxon
Polarizability26.84 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference

Predicted Kovats Retention Indices


MetaboliteSMILESKovats RI ValueColumn TypeReference
ValerylcarnitineCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C2602.1Standard polar33892256
ValerylcarnitineCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C1457.3Standard non polar33892256
ValerylcarnitineCCCCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C1691.0Semi standard non polar33892256


Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Valerylcarnitine,1TMS,isomer #1CCCCC(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C1717.4Semi standard non polar33892256
Valerylcarnitine,1TBDMS,isomer #1CCCCC(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C1943.5Semi standard non polar33892256

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Valerylcarnitine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Valerylcarnitine 10V, Positive-QTOFsplash10-0002-0090000000-a919a7a98ddb53d7bb2b2021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Valerylcarnitine 20V, Positive-QTOFsplash10-000j-9050000000-87beaf2642b8fc410f0c2021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Valerylcarnitine 40V, Positive-QTOFsplash10-000i-9000000000-e9262cbaff8cb4ad0ba62021-09-25Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationsNot Available
Normal Concentrations
BloodDetected and Quantified<0.400 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.14 +/- 0.06 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.040-0.260 uMAdult (>18 years old)Both
BloodDetected and Quantified<0.600 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified0.16(0.04) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.46 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified0.14 +/- 0.04 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<0.310 uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.013 +/- 0.006 uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected and Quantified0.21 +/- 0.09 nmol/g wet fecesAdult (>18 years old)Both
FecesDetected and Quantified0.14 +/- 0.06 nmol/g wet fecesAdult (>18 years old)Both
UrineDetected and Quantified0.220 (0.037-0.440) umol/mmol creatinineAdult (>18 years old)Both
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified<3.0 umol/mmol creatinineNewborn (0-30 days old)Not SpecifiedNormal details
UrineDetected and Quantified0.9187 +/- 0.4397 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    • Analysis of 30 no...
UrineDetected and Quantified0.02-0.29 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.04(0.02-0.25) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified0.05(0.02-0.3) umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
Abnormal Concentrations
BloodDetected and Quantified27.300-48.200 uMNot SpecifiedNot SpecifiedIsovaleric acidemia details
BloodDetected and Quantified0.2(0.06) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified0.22(0.08) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected and Quantified0.0845 (0.0722) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified0.1179 (0.0241) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified0.510-2.0800 uMNewborn (0-30 days old)Both2-Methylbutyryl-CoA dehydrogenase deficiency (SBACDD) details
BloodDetected and Quantified0.34 uMChildren (1-13 years old)Male2-Methylbutyryl-CoA dehydrogenase deficiency (SBACDD) details
BloodDetected and Quantified0.089 +/- 0.041 uMChildren (1-13 years old)Both
    • Metabolomics reve...
BloodDetected and Quantified0.096 +/- 0.031 uMChildren (1-13 years old)Both
    • Metabolomics reve...
UrineDetected and Quantified0.6537 +/- 0.7142 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
UrineDetected and Quantified0.4795 +/- 0.3669 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Analysis of 30 no...
Associated Disorders and Diseases
Disease References
  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 ]
  1. 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.
Isovaleric acidemia
  1. Matern D, He M, Berry SA, Rinaldo P, Whitley CB, Madsen PP, van Calcar SC, Lussky RC, Andresen BS, Wolff JA, Vockley J: Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry. Pediatrics. 2003 Jul;112(1 Pt 1):74-8. [PubMed:12837870 ]
Short/branched chain acyl-CoA dehydrogenase deficiency
  1. Sass JO, Ensenauer R, Roschinger W, Reich H, Steuerwald U, Schirrmacher O, Engel K, Haberle J, Andresen BS, Megarbane A, Lehnert W, Zschocke J: 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism. Mol Genet Metab. 2008 Jan;93(1):30-5. doi: 10.1016/j.ymgme.2007.09.002. Epub 2007 Oct 22. [PubMed:17945527 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
  • 601665 (Obesity)
  • 243500 (Isovaleric acidemia)
  • 610006 (Short/branched chain acyl-CoA dehydrogenase deficiency)
  • 610247 (Eosinophilic esophagitis)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB029301
KNApSAcK IDNot Available
Chemspider ID17353685
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound16226475
PDB IDNot Available
ChEBI ID85095
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000850
Good Scents IDNot Available
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  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 ]
  2. Zordoky BN, Sung MM, Ezekowitz J, Mandal R, Han B, Bjorndahl TC, Bouatra S, Anderson T, Oudit GY, Wishart DS, Dyck JR: Metabolomic fingerprint of heart failure with preserved ejection fraction. PLoS One. 2015 May 26;10(5):e0124844. doi: 10.1371/journal.pone.0124844. eCollection 2015. [PubMed:26010610 ]
  3. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. Zhang X, Li Y, Liang Y, Sun P, Wu X, Song J, Sun X, Hong M, Gao P, Deng D: Distinguishing Intracerebral Hemorrhage from Acute Cerebral Infarction through Metabolomics. Rev Invest Clin. 2017 Nov-Dec;69(6):319-328. doi: 10.24875/RIC.17002348. [PubMed:29265114 ]
  11. Mihalik SJ, Goodpaster BH, Kelley DE, Chace DH, Vockley J, Toledo FG, DeLany JP: Increased levels of plasma acylcarnitines in obesity and type 2 diabetes and identification of a marker of glucolipotoxicity. Obesity (Silver Spring). 2010 Sep;18(9):1695-700. doi: 10.1038/oby.2009.510. Epub 2010 Jan 28. [PubMed:20111019 ]
  12. Chao de la Barca JM, Rondet-Courbis B, Ferre M, Muller J, Buisset A, Leruez S, Plubeau G, Mace T, Moureauzeau L, Chupin S, Tessier L, Blanchet O, Lenaers G, Procaccio V, Mirebeau-Prunier D, Simard G, Gohier P, Milea D, Reynier P: A Plasma Metabolomic Profiling of Exudative Age-Related Macular Degeneration Showing Carnosine and Mitochondrial Deficiencies. J Clin Med. 2020 Feb 27;9(3). pii: jcm9030631. doi: 10.3390/jcm9030631. [PubMed:32120889 ]
  13. Sun Y, Gao HY, Fan ZY, He Y, Yan YX: Metabolomics Signatures in Type 2 Diabetes: A Systematic Review and Integrative Analysis. J Clin Endocrinol Metab. 2020 Apr 1;105(4). pii: 5645632. doi: 10.1210/clinem/dgz240. [PubMed:31782507 ]
  14. Dambrova M, Makrecka-Kuka M, Kuka J, Vilskersts R, Nordberg D, Attwood MM, Smesny S, Sen ZD, Guo AC, Oler E, Tian S, Zheng J, Wishart DS, Liepinsh E, Schioth HB: Acylcarnitines: Nomenclature, Biomarkers, Therapeutic Potential, Drug Targets, and Clinical Trials. Pharmacol Rev. 2022 Jul;74(3):506-551. doi: 10.1124/pharmrev.121.000408. [PubMed:35710135 ]
  15. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.