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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-12-07 01:19:31 UTC
HMDB IDHMDB0000752
Secondary Accession Numbers
  • HMDB00752
Metabolite Identification
Common NameMethylglutaric acid
DescriptionMethylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239 ). Methylglutaric acid is also found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, another inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
beta-Methylglutaric acidChEBI
b-MethylglutarateGenerator
b-Methylglutaric acidGenerator
beta-MethylglutarateGenerator
β-methylglutarateGenerator
β-methylglutaric acidGenerator
MethylglutarateGenerator
3-Methyl-glutarateHMDB
3-Methyl-glutaric acidHMDB
3-MethylglutarateHMDB
3-Methylglutaric acidHMDB
3-MethylpentanedioateHMDB
3-Methylpentanedioic acidHMDB
b-Methyl-glutaric acidHMDB
beta-Methyl-glutaric acidHMDB
Chemical FormulaC6H10O4
Average Molecular Weight146.1412
Monoisotopic Molecular Weight146.057908808
IUPAC Name3-methylpentanedioic acid
Traditional Namemethylglutaric acid
CAS Registry Number626-51-7
SMILES
CC(CC(O)=O)CC(O)=O
InChI Identifier
InChI=1S/C6H10O4/c1-4(2-5(7)8)3-6(9)10/h4H,2-3H2,1H3,(H,7,8)(H,9,10)
InChI KeyXJMMNTGIMDZPMU-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentMethyl-branched fatty acids
Alternative Parents
Substituents
  • Methyl-branched fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

  Cell and elements:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Cellular process:

    Chemical reaction:

    Biochemical process:

    Biochemical pathway:

Role

Indirect biological role:

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point80 - 82 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility36.9 g/LALOGPS
logP0.33ALOGPS
logP0.33ChemAxon
logS-0.6ALOGPS
pKa (Strongest Acidic)3.91ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity32.69 m³·mol⁻¹ChemAxon
Polarizability13.88 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-0910000000-81203203246ac374e1aaView in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-00xr-2930000000-8a7588ab0eaf67584f92View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0930000000-5140c0f39766a00d8482View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0930000000-fbddcb97da0458138e6bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-81203203246ac374e1aaView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00xr-2930000000-8a7588ab0eaf67584f92View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-1a70e49844a70861269aView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-000i-9400000000-4f63fb4e9602cbd17564View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dr-9320000000-32ac86c797d0bc1d6ae4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0udi-0900000000-eb243e6803451ac1e484View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4i-9000000000-f5d1e90d94d8c01d6413View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0aor-9200000000-5a9c1dfa7ad59a2bb309View in MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-0udi-0900000000-1b870c5514c1893d28a0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-000i-6900000000-a6e3d73c4975ca021d59View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-004j-1900000000-0d7094ff6083f2cbfb11View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0ftu-9400000000-3e6419aef7a791fa1444View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-a7c4a49f42f193d900b0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-1900000000-1eb2402c520f7d009743View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0f92-2900000000-32ccc4d90c40abb61344View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9200000000-5b5c0b72c3ec914ca4e7View in MoNA
MSMass Spectrum (Electron Ionization)splash10-0frf-9100000000-a7e54dd57900b7dde601View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Feces
  • Urine
Tissue Location
  • Prostate
PathwaysNot Available
NameSMPDB/PathwhizKEGG
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    UrineDetected and Quantified<5 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified0.5 +/- 0.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified1.0 - 6.5 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
    UrineDetected and Quantified0.75-1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified0.54-0.80 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified<3.9 umol/mmol creatinineChildren (1 - 18 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified1.6 (1.3-2.0) umol/mmol creatinineAdult (>18 years old)BothNormal
      • Geigy Scientific ...
    details
    UrineDetected and Quantified0.1 (0.1-9.7) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
    UrineDetected and Quantified0.1 (0.1-0.1) umol/mmol creatinineInfant (0-1 year old)BothNormal details
    UrineDetected and Quantified1.0 (0.1-2.8) umol/mmol creatinineChildren (1-13 years old)Both
    Normal
    details
    UrineDetected and Quantified1.1 (0.1-2.0) umol/mmol creatinineChildren (1-13 years old)Both
    Normal
    details
    UrineDetected and Quantified0.6 (0.1-2.1) umol/mmol creatinineAdolescent (13-18 years old)Both
    Normal
    details
    UrineDetected and Quantified3.1 (2.6-3.6) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    UrineDetected and Quantified348 umol/mmol creatinineNewborn (0-30 days old)Male3-hydroxy-3-methylglutaric- coenzyme A lyase deficiency details
    UrineDetected and Quantified622 umol/mmol creatinineAdult (>18 years old)Female3-Hydroxy-3-Methylglutaryl- CoA Lyase Deficiency details
    UrineDetected and Quantified12-15 umol/mmol creatinineAdult (>18 years old)Female3-methylglutaconic aciduria type I details
    UrineDetected and Quantified3.5 (0.0-7.0) umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified507.0 (14.0-1000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
      • MetaGene: Metabol...
    details
    Associated Disorders and Diseases
    Disease References
    3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    3-Methylglutaconic aciduria type I
    1. Engelke UF, Kremer B, Kluijtmans LA, van der Graaf M, Morava E, Loupatty FJ, Wanders RJ, Moskau D, Loss S, van den Bergh E, Wevers RA: NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR Biomed. 2006 Apr;19(2):271-8. [PubMed:16541463 ]
    2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    Associated OMIM IDs
    • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    • 250950 (3-Methylglutaconic aciduria type I)
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB022224
    KNApSAcK IDNot Available
    Chemspider ID11781
    KEGG Compound IDNot Available
    BioCyc IDCPD-516
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID5720
    PubChem Compound12284
    PDB IDNot Available
    ChEBI ID68566
    References
    Synthesis ReferenceKomppa, Gust. b-Methylglutaric acid. Ann. acad. sci. Fennicae (1930), 30(Ser. A;No. 9), 5 pp.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
    2. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
    3. Truscott RJ, Halpern B, Wysocki SJ, Hahnel R, Wilcken B: Studies on a child suspected of having a dficiency in 3-hydroxy-3-methylglutaryl-Co A lyase. Clin Chim Acta. 1979 Jul 2;95(1):11-16. [PubMed:509721 ]
    4. Sheffer RN, Zlotogora J, Elpeleg ON, Raz J, Ben-Ezra D: Behr's syndrome and 3-methylglutaconic aciduria. Am J Ophthalmol. 1992 Oct 15;114(4):494-7. [PubMed:1384336 ]
    5. Duran M, Beemer FA, Tibosch AS, Bruinvis L, Ketting D, Wadman SK: Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism. J Pediatr. 1982 Oct;101(4):551-4. [PubMed:6181239 ]