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Showing metabocard for 3-Methylglutarylcarnitine (HMDB0000552)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesXML
Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-03-23 17:40:32 UTC
HMDB IDHMDB0000552
Secondary Accession Numbers
  • HMDB00552
Metabolite Identification
Common Name3-Methylglutarylcarnitine
Description3-Methylglutarylcarnitine belongs to the class of organic compounds known as acylcarnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond 3-Methylglutarylcarnitine is an extremely weak basic (essentially neutral) compound (based on its pKa). 3-Methylglutarylcarnitine is a diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. It is also identified in the urine of patients with Reye-like syndrome (PMID: 3958190 , 10927963 ).
Structure
Data?1584985232
MOLSDFPDBSMILESInChI
Synonyms
ValueSource
(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acidGenerator
3-MethylglutarylcarnitineHMDB
MethylglutarylcarnitineHMDB
O-3-MethylglutarylcarnitineHMDB
Chemical FormulaC13H23NO6
Average Molecular Weight289.328
Monoisotopic Molecular Weight289.152537465
IUPAC Name(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoate
Traditional Name(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylammonio)butanoate
CAS Registry Number102673-95-0
SMILES
C[C@H](CC(O)=O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C13H23NO6/c1-9(5-11(15)16)6-13(19)20-10(7-12(17)18)8-14(2,3)4/h9-10H,5-8H2,1-4H3,(H-,15,16,17,18)/t9-,10-/m1/s1
InChI KeyHFCPFJNSBPQJDP-NXEZZACHSA-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
  • 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
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 Solubility0.066 g/LALOGPS
logP-1.8ALOGPS
logP-3.9ChemAxon
logS-3.7ALOGPS
pKa (Strongest Acidic)3.42ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area103.73 ŲChemAxon
Rotatable Bond Count10ChemAxon
Refractivity92.68 m³·mol⁻¹ChemAxon
Polarizability29.55 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.0000-0.140 uMNot SpecifiedNot SpecifiedNormal details
BloodExpected but not Quantified Not AvailableNot Available
Normal
    		 details
    BloodDetected and Quantified0.020-0.060 uMAdult (>18 years old)BothNormal details
    FecesDetected and Quantified0.24 +/- 0.1 nmol/g wet fecesAdult (>18 years old)Both
    Normal    		 details
    FecesDetected and Quantified1.1 +/- 0.65 nmol/g wet fecesAdult (>18 years old)Both
    Normal    		 details
    SalivaDetected and Quantified0.088 +/- 0.011 uMAdult (>18 years old)BothNormal
      • Zerihun T. Dame, ...
    		 details
    UrineDetected and Quantified0.03-0.13 umol/mmol creatinineNewborn (0-30 days old)BothNormal
      • López Hernández Y...
    		 details
    UrineDetected and Quantified0.05(0.03-0.13) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal
      • López Hernández Y...
    		 details
    UrineDetected and Quantified0.06(0.03-0.13) umol/mmol creatinineNewborn (0-30 days old)MaleNormal
      • López Hernández Y...
    		 details
    UrineDetected and Quantified0.030 (0.016-0.052) umol/mmol creatinineAdult (>18 years old)Both
    Normal    		 details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified0.76 uMNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    BloodDetected and Quantified0.049 (0.0327) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
    Associated Disorders and Diseases
    Disease References
    3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    1. Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
    Associated OMIM IDs
    • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FooDB IDFDB022112
    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
    References
    Synthesis ReferenceJooste, S.; Erasmus, E.; Mienie, L. J.; de Wet, W. J.; Gibson, K. M. The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. Clinica Chimica Acta (1994), 230(1), 1-8.
    Material Safety Data Sheet (MSDS)Not Available
    General References
    1. Roe CR, Millington DS, Maltby DA: Identification of 3-methylglutarylcarnitine. A new diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. J Clin Invest. 1986 Apr;77(4):1391-4. [PubMed:3958190 ]
    2. Lee C, Tsai FJ, Wu JY, Peng CT, Tsai CH, Hwu WL, Wang TR, Millington DS: 3-hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case. Acta Paediatr Taiwan. 1999 Nov-Dec;40(6):445-7. [PubMed:10927963 ]
    3. Jooste S, Erasmus E, Mienie LJ, de Wet WJ, Gibson KM: The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. Clin Chim Acta. 1994 Oct 14;230(1):1-8. [PubMed:7850987 ]