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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:44:27 UTC
HMDB IDHMDB0000752
Secondary Accession Numbers
  • HMDB00752
Metabolite Identification
Common NameMethylglutaric acid
DescriptionMethylglutaric acid, also known as b-methylglutarate or 3-methylpentanedioate, belongs to the class of organic compounds 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. Methylglutaric acid is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. Methylglutaric acid is a potentially toxic compound.
Structure
Data?1563860667
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
Description belongs to the class of organic compounds 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.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
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

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

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
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-0910000000-81203203246ac374e1aaJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-00xr-2930000000-8a7588ab0eaf67584f92JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0930000000-5140c0f39766a00d8482JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0930000000-fbddcb97da0458138e6bJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-81203203246ac374e1aaJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00xr-2930000000-8a7588ab0eaf67584f92JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-1a70e49844a70861269aJSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-000i-9400000000-4f63fb4e9602cbd17564JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dr-9320000000-32ac86c797d0bc1d6ae4JSpectraViewer
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0udi-0900000000-eb243e6803451ac1e484JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4i-9000000000-f5d1e90d94d8c01d6413JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0aor-9200000000-5a9c1dfa7ad59a2bb309JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-0udi-0900000000-1b870c5514c1893d28a0JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-000i-6900000000-a6e3d73c4975ca021d59JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-004j-1900000000-0d7094ff6083f2cbfb11JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0ftu-9400000000-3e6419aef7a791fa1444JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-a7c4a49f42f193d900b0JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-1900000000-1eb2402c520f7d009743JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0f92-2900000000-32ccc4d90c40abb61344JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9200000000-5b5c0b72c3ec914ca4e7JSpectraViewer
MSMass Spectrum (Electron Ionization)splash10-0frf-9100000000-a7e54dd57900b7dde601JSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR13C NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    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 Quantified<500 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
    UrineDetected and Quantified<7 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal 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 Quantified<5 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
    UrineDetected and Quantified0.1 (0.1-9.7) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
    UrineDetected and Quantified0.1 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
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal 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 Quantified1400 umol/mmol creatinineChildren (1-13 years old)Male3-Methylglutaconic Aciduria (Type V) details
    UrineDetected and Quantified348 umol/mmol creatinineNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    UrineDetected and Quantified622 umol/mmol creatinineAdult (>18 years old)Female3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    UrineDetected but not Quantified Adult (>18 years old)BothBladder cancer 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
    Colorectal cancer
    1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
    2. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
    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 ]
    2. Bischof F, Nagele T, Wanders RJ, Trefz FK, Melms A: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in an adult with leukoencephalopathy. Ann Neurol. 2004 Nov;56(5):727-30. [PubMed:15505778 ]
    3. 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.
    3-Methylglutaconic Aciduria type V
    1. Ojala T, Polinati P, Manninen T, Hiippala A, Rajantie J, Karikoski R, Suomalainen A, Tyni T: New mutation of mitochondrial DNAJC19 causing dilated and noncompaction cardiomyopathy, anemia, ataxia, and male genital anomalies. Pediatr Res. 2012 Oct;72(4):432-7. doi: 10.1038/pr.2012.92. Epub 2012 Jul 13. [PubMed:22797137 ]
    Associated OMIM IDs
    • 114500 (Colorectal cancer)
    • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    • 250950 (3-Methylglutaconic aciduria type I)
    • 610198 (3-Methylglutaconic Aciduria type V)
    DrugBank IDNot Available
    Phenol Explorer Compound 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
    Food Biomarker OntologyNot Available
    VMH IDNot Available
    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. 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 ]
    2. 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 ]
    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. 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 ]
    5. 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 ]