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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-05-22 14:17:36 UTC
Update Date2019-07-23 05:45:18 UTC
HMDB IDHMDB0002095
Secondary Accession Numbers
  • HMDB0062496
  • HMDB0062592
  • HMDB02095
  • HMDB62496
  • HMDB62592
Metabolite Identification
Common NameMalonylcarnitine
DescriptionMalonylcarnitine is a metabolite that accumulates with specific disruption of fatty-acid oxidation caused by the impaired entry of long-chain acylcarnitine esters into the mitochondria and failure of the mitochondrial respiratory chain at complex 11 and malonyl-CoA decarboxylase (EC 4.1.1.9) deficiency (OMIM: 248360 ). Malonylcarnitine has also been found to accumulate in some newborns with medium-chain acyl-CoA dehydrogenase (EC 1.3.99.3) deficiency (OMIM: 201450 ). (PMID: 11558490 , 15303003 , 12651823 ).
Structure
Data?1563860718
Synonyms
ValueSource
Malonyl-CarnitinHMDB
Malonyl-CarnitineHMDB
Malonyl-L-carnitineHMDB
MalonylcarnitineHMDB
O-Malonyl-L-carnitineHMDB
O-MalonylcarnitineHMDB
Chemical FormulaC10H17NO6
Average Molecular Weight247.247
Monoisotopic Molecular Weight247.105587271
IUPAC Name(3R)-3-[(2-carboxyacetyl)oxy]-4-(trimethylazaniumyl)butanoate
Traditional Name(3R)-3-[(2-carboxyacetyl)oxy]-4-(trimethylammonio)butanoate
CAS Registry Number910825-21-7
SMILES
C[N+](C)(C)C[C@@H](CC([O-])=O)OC(=O)CC(O)=O
InChI Identifier
InChI=1S/C10H17NO6/c1-11(2,3)6-7(4-8(12)13)17-10(16)5-9(14)15/h7H,4-6H2,1-3H3,(H-,12,13,14,15)/t7-/m1/s1
InChI KeyZGNBLKBZJBJFDG-SSDOTTSWSA-N
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
  • Quaternary ammonium salt
  • Tetraalkylammonium salt
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organic salt
  • Organopnictogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

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 Solubility0.34 g/LALOGPS
logP-2ALOGPS
logP-4.6ChemAxon
logS-3ALOGPS
pKa (Strongest Acidic)3.47ChemAxon
pKa (Strongest Basic)-7.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area103.73 ŲChemAxon
Rotatable Bond Count8ChemAxon
Refractivity78.98 m³·mol⁻¹ChemAxon
Polarizability23.68 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9110000000-c695dab07ed816875c72JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-03di-0900000000-9b579c570aab7c7d3a21JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0ik9-0900000000-e37d3def2de1af5bd2f5JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0ue9-8900000000-cf37f0a721cd52d86e8eJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0r0c-9200000000-a11a4872036f3c33d25aJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0a4l-9000000000-2402e260a330ff1772a1JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , positivesplash10-0w29-6900000000-d0136248acdd706e650bJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0ik9-2900000000-61732bb10307f2b183caJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0zg0-9400000000-15c3db3324f1d46d3c7cJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0ik9-2900000000-ee79f10dce9fc09246ecJSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.030-0.090 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified0.1 (0 - 0.2) uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified<0.30 uMNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.02 (0.01-0.04) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified2.7 (1.6 - 3.8) uMNewborn (0-30 days old)Not SpecifiedMalonyl-CoA decarboxylase deficiency details
BloodDetected and Quantified0.2 (0.13 - 0.3) uMInfant (0-1 year old)Not SpecifiedMalonyl-CoA decarboxylase deficiency details
BloodDetected and Quantified46.8 (24.8) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified27.8 (20.0) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified50.65(14.14) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
Associated Disorders and Diseases
Disease References
Malonyl-Coa decarboxylase deficiency
  1. Santer R, Fingerhut R, Lassker U, Wightman PJ, Fitzpatrick DR, Olgemoller B, Roscher AA: Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency. Clin Chem. 2003 Apr;49(4):660-2. [PubMed:12651823 ]
  2. Ficicioglu C, Chrisant MR, Payan I, Chace DH: Cardiomyopathy and hypotonia in a 5-month-old infant with malonyl-coa decarboxylase deficiency: potential for preclinical diagnosis with expanded newborn screening. Pediatr Cardiol. 2005 Nov-Dec;26(6):881-3. [PubMed:16078122 ]
Late-onset preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Associated OMIM IDs
  • 248360 (Malonyl-Coa decarboxylase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022843
KNApSAcK IDNot Available
Chemspider ID34999703
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6484
PubChem Compound91825606
PDB IDNot Available
ChEBI ID85470
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Johnson DW, Trinh MU: Stability of malonylcarnitine and glutarylcarnitine in stored blood spots. J Inherit Metab Dis. 2004;27(6):789-90. [PubMed:15617193 ]
  2. Santer R, Fingerhut R, Lassker U, Wightman PJ, Fitzpatrick DR, Olgemoller B, Roscher AA: Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency. Clin Chem. 2003 Apr;49(4):660-2. [PubMed:12651823 ]
  3. Napolitano N, Wiley V, Pitt JJ: Pseudo-glutarylcarnitinaemia in medium-chain acyl-CoA dehydrogenase deficiency detected by tandem mass spectrometry newborn screening. J Inherit Metab Dis. 2004;27(4):465-71. [PubMed:15303003 ]
  4. Wolf A, Weir P, Segar P, Stone J, Shield J: Impaired fatty acid oxidation in propofol infusion syndrome. Lancet. 2001 Feb 24;357(9256):606-7. [PubMed:11558490 ]
  5. Ficicioglu C, Chrisant MR, Payan I, Chace DH: Cardiomyopathy and hypotonia in a 5-month-old infant with malonyl-coa decarboxylase deficiency: potential for preclinical diagnosis with expanded newborn screening. Pediatr Cardiol. 2005 Nov-Dec;26(6):881-3. [PubMed:16078122 ]
  6. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.