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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-12-07 01:17:16 UTC
HMDB IDHMDB0000522
Secondary Accession Numbers
  • HMDB00522
Metabolite Identification
Common Name3-Methylglutaconic acid
Description3-Methylglutaconic acid is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism (PMID: 7603789 ). 3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. 3-Methylglutaconic acid accumulates in patients with a deficiency of this enzyme and a large amount of 3-methylglutaconic acid appear in urine (Wikipedia). 3-Methylglutaconic acid is also found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, which is also an inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
3-MethylglutaconateHMDB
b-MethylglutaconateHMDB
b-Methylglutaconic acidHMDB
beta-MethylglutaconateHMDB
beta-Methylglutaconic acidHMDB
Chemical FormulaC6H8O4
Average Molecular Weight144.1253
Monoisotopic Molecular Weight144.042258744
IUPAC Name(2E)-3-methylpent-2-enedioic acid
Traditional Nameβ-methylglutaconic acid
CAS Registry Number5746-90-7
SMILES
C\C(CC(O)=O)=C/C(O)=O
InChI Identifier
InChI=1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+
InChI KeyWKRBKYFIJPGYQC-DUXPYHPUSA-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
  • Unsaturated 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:

  Cell and elements:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Cellular process:

    Biochemical pathway:

    Chemical reaction:

    Biochemical process:

Physiological effect

Health effect:

  Health condition:

    Metabolism and nutrition disorders:

Role

Indirect biological 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 Solubility8.69 g/LALOGPS
logP0.37ALOGPS
logP0.29ChemAxon
logS-1.2ALOGPS
pKa (Strongest Acidic)3.85ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity33.51 m³·mol⁻¹ChemAxon
Polarizability13.27 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0015-9200000000-ff306cede85d252e0d4cView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00di-9340000000-0b493822a103307b8f66View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-002b-2900000000-b6862aeffb1aa9fae1d0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-9300000000-7ab01dfb0b86abdac817View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-3692ceff17a888ae8836View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0006-5900000000-dc4d4a80a164f3fd1feaView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0007-8900000000-7ece848c8011e4a20200View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a7l-9300000000-8b54483c15a17429b9f8View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified<10 uMAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<10 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified<25 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified1.0 - 6.5 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<12.42 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified6.2 (2.8-8.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified10.707 +/- 4.243 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified7.5 (3.4-9.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified15.86 +/- 6.052 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified125 uMAdult (>18 years old)Female3-methylglutaconic aciduria type I details
UrineDetected and Quantified750.0 (500.0-1000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Methylglutaconic Aciduria (TYPE I)
    • MetaGene: Metabol...
details
UrineDetected and Quantified0-230 umol/mmol creatinineInfant (0-1 year old)Male3-methylglutaconic aciduria type II, X-linked details
UrineDetected and Quantified3714 umol/mmol creatinineAdult (>18 years old)Female3-Hydroxy-3-Methylglutaryl- CoA Lyase Deficiency details
UrineDetected and Quantified32-51 umol/mmol creatinineAdult (>18 years old)Female3-methylglutaconic aciduria type I details
UrineDetected and Quantified62-90 umol/mmol creatinineAdult (>18 years old)Female3-methylglutaconic aciduria type I details
UrineDetected and Quantified94-141 umol/mmol creatinineAdult (>18 years old)Not Specified
3-Methylglutaconic Aciduria (TYPE I)
details
UrineDetected and Quantified570 umol/mmol creatinineChildren (1-13 years old)Not Specified3-Methylglutaconic Aciduria (TYPE I) details
UrineDetected and Quantified10.714 +/- 4.929 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified12.053 +/- 6.469 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified14.626 +/- 7.998 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified4.5 (0.00-9.0) umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    • MetaGene: Metabol...
details
UrineDetected and Quantified17.038 +/- 8.547 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified5070.0 (140.0-10000.0) 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.
3-methylglutaconic aciduria type II, X-linked
  1. Cantlay AM, Shokrollahi K, Allen JT, Lunt PW, Newbury-Ecob RA, Steward CG: Genetic analysis of the G4.5 gene in families with suspected Barth syndrome. J Pediatr. 1999 Sep;135(3):311-5. [PubMed:10484795 ]
Associated OMIM IDs
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 250950 (3-Methylglutaconic aciduria type I)
  • 302060 (3-methylglutaconic aciduria type II, X-linked)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022090
KNApSAcK IDNot Available
Chemspider ID1267861
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia Link3-Methylglutaconic acid
METLIN ID5507
PubChem Compound1551553
PDB IDNot Available
ChEBI ID37245
References
Synthesis ReferenceAoyama, Takayuki; Kato, Kazuo; Shimada, Nobuyoshi; Fujii, Akio; Takita, Tomohisa. Production of 3-methylglutaconic acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Mitchell GA, Jakobs C, Gibson KM, Robert MF, Burlina A, Dionisi-Vici C, Dallaire L: Molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase deficiency. Prenat Diagn. 1995 Aug;15(8):725-9. [PubMed:7479590 ]
  2. Holme E, Greter J, Jacobson CE, Larsson NG, Lindstedt S, Nilsson KO, Oldfors A, Tulinius M: Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria. Pediatr Res. 1992 Dec;32(6):731-5. [PubMed:1287564 ]
  3. Costeff H, Elpeleg O, Apter N, Divry P, Gadoth N: 3-Methylglutaconic aciduria in "optic atrophy plus". Ann Neurol. 1993 Jan;33(1):103-4. [PubMed:8494328 ]
  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. Kelley RI: Quantification of 3-methylglutaconic acid in urine, plasma, and amniotic fluid by isotope-dilution gas chromatography/mass spectrometry. Clin Chim Acta. 1993 Nov 15;220(2):157-64. [PubMed:8111960 ]
  6. Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. [PubMed:7603789 ]