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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2009-11-03 15:30:49 UTC
Update Date2022-10-24 19:44:11 UTC
HMDB IDHMDB0013133
Secondary Accession Numbers
  • HMDB13133
Metabolite Identification
Common NameMethylmalonylcarnitine
DescriptionMethylmalonylcarnitine is an acylcarnitine. More specifically, it is an methylmalonic 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. Methylmalonylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine methylmalonylcarnitine 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 methylmalonylcarnitine is elevated in the blood or plasma of individuals with methylmalonyl-coa epimerase deficiency/methylmalonic acidemia (PMID: 27699154 , PMID: 17279485 ), familial mediterranean fever (PMID: 29900937 ), and cobalamin C deficiency (PMID: 25367534 ). It is also decreased in the blood or plasma of individuals with melanoma (PMID: 30830422 ) and intracerebral hemorrhage (PMID: 29265114 ). Methylmalonylcarnitine is elevated in the urine of individuals with methylmalonyl-coa epimerase deficiency/methylmalonic acidemia (PMID: 27699154 , PMID: 17279485 ). Carnitine acetyltransferase (CrAT, EC:2.3.1.7) 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 ).
Structure
Data?1614293816
Synonyms
ValueSource
(2R)-3-{[(2R)-1-carboxy-3-(trimethylazaniumyl)propan-2-yl]oxy}-2-methyl-3-oxopropanoic acidHMDB
Methylmalonyl-L-carnitineHMDB
MethylmalonylcarnitineHMDB
Chemical FormulaC11H20NO6
Average Molecular Weight262.281
Monoisotopic Molecular Weight262.128513788
IUPAC Name(3R)-3-{[(2R)-2-carboxy-2-methylacetyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Name(3R)-3-{[(2R)-2-carboxy-2-methylacetyl]oxy}-4-(trimethylammonio)butanoate
CAS Registry Number256928-70-8
SMILES
C[C@H](C(O)=O)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C11H19NO6/c1-7(10(15)16)11(17)18-8(5-9(13)14)6-12(2,3)4/h7-8H,5-6H2,1-4H3,(H-,13,14,15,16)/p+1/t7-,8-/m1/s1
InChI KeyXROYFEWIXXCPAW-HTQZYQBOSA-O
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
Substituents
  • Acyl-carnitine
  • Tricarboxylic acid or derivatives
  • 1,3-dicarbonyl compound
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organic salt
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect
Disposition
ProcessNot Available
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility0.27 g/LALOGPS
logP-1.6ALOGPS
logP-4ChemAxon
logS-3.1ALOGPS
pKa (Strongest Acidic)3.53ChemAxon
pKa (Strongest Basic)-7.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area103.73 ŲChemAxon
Rotatable Bond Count8ChemAxon
Refractivity83.56 m³·mol⁻¹ChemAxon
Polarizability25.62 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DeepCCS[M+H]+160.23930932474
DeepCCS[M-H]-156.77630932474
DeepCCS[M-2H]-192.99330932474
DeepCCS[M+Na]+168.64130932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
MethylmalonylcarnitineC[C@H](C(O)=O)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C2950.1Standard polar33892256
MethylmalonylcarnitineC[C@H](C(O)=O)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C1571.2Standard non polar33892256
MethylmalonylcarnitineC[C@H](C(O)=O)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C1879.9Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Methylmalonylcarnitine,1TMS,isomer #1C[C@@H](C(=O)O[C@H](CC(=O)O)C[N+](C)(C)C)C(=O)O[Si](C)(C)C1784.6Semi standard non polar33892256
Methylmalonylcarnitine,1TMS,isomer #2C[C@H](C(=O)O)C(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C1779.0Semi standard non polar33892256
Methylmalonylcarnitine,2TMS,isomer #1C[C@@H](C(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C)C(=O)O[Si](C)(C)C1845.3Semi standard non polar33892256
Methylmalonylcarnitine,1TBDMS,isomer #1C[C@@H](C(=O)O[C@H](CC(=O)O)C[N+](C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2015.0Semi standard non polar33892256
Methylmalonylcarnitine,1TBDMS,isomer #2C[C@H](C(=O)O)C(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C2004.0Semi standard non polar33892256
Methylmalonylcarnitine,2TBDMS,isomer #1C[C@@H](C(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2292.8Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Methylmalonylcarnitine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylmalonylcarnitine 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 - Methylmalonylcarnitine 10V, Positive-QTOFsplash10-03di-0090000000-48d875468ed156af31be2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylmalonylcarnitine 20V, Positive-QTOFsplash10-01p9-9050000000-49b1ecee6d2effc4a3652021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylmalonylcarnitine 40V, Positive-QTOFsplash10-000i-9000000000-e9262cbaff8cb4ad0ba62021-09-22Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot Available
Normal
      Not Available
details
BloodDetected and Quantified<0.110 uMNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified0.09 (0.05-0.16) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.0602 (0.0527) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB112307
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem CompoundNot Available
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. Rahmandar MH, Bawcom A, Romano ME, Hamid R: Cobalamin C deficiency in an adolescent with altered mental status and anorexia. Pediatrics. 2014 Dec;134(6):e1709-14. doi: 10.1542/peds.2013-2711. Epub 2014 Nov 3. [PubMed:25367534 ]
  11. Waters PJ, Thuriot F, Clarke JT, Gravel S, Watkins D, Rosenblatt DS, Levesque S: Methylmalonyl-coA epimerase deficiency: A new case, with an acute metabolic presentation and an intronic splicing mutation in the MCEE gene. Mol Genet Metab Rep. 2016 Sep 24;9:19-24. doi: 10.1016/j.ymgmr.2016.09.001. eCollection 2016 Dec. [PubMed:27699154 ]
  12. Maeda Y, Ito T, Suzuki A, Kurono Y, Ueta A, Yokoi K, Sumi S, Togari H, Sugiyama N: Simultaneous quantification of acylcarnitine isomers containing dicarboxylic acylcarnitines in human serum and urine by high-performance liquid chromatography/electrospray ionization tandem mass spectrometry. Rapid Commun Mass Spectrom. 2007;21(5):799-806. doi: 10.1002/rcm.2905. [PubMed:17279485 ]
  13. Bayci AWL, Baker DA, Somerset AE, Turkoglu O, Hothem Z, Callahan RE, Mandal R, Han B, Bjorndahl T, Wishart D, Bahado-Singh R, Graham SF, Keidan R: Metabolomic identification of diagnostic serum-based biomarkers for advanced stage melanoma. Metabolomics. 2018 Aug 3;14(8):105. doi: 10.1007/s11306-018-1398-9. [PubMed:30830422 ]
  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.