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Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-10-09 20:59:55 UTC
Secondary Accession Numbers
  • HMDB0001657
  • HMDB0061185
  • HMDB00620
  • HMDB01657
  • HMDB61185
Metabolite Identification
Common NameGlutaconic acid
DescriptionGlutaconic acid is related to the fully saturated glutaric acid and belongs to the class of compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups. Glutaconic acid is soluble (in water) and a weakly acidic compound (based on its pKa). Glutaconic acid has been detected in the urine of individuals with inborn errors of metabolism. When present in sufficiently high levels, glutaconic acid can act as an acidogen, a neurotoxin, and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A neurotoxin is a compound that is toxic to neural tissues and cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaconic acid are associated with glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1). GA1 is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC, GCDH). Excessive levels of their intermediate breakdown products (e.g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs), but particularly the basal ganglia. GA1 is associated with a risk for intracranial and retinal hemorrhage, and non-specific white matter changes. Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Other symptoms include spasticity (increased muscle tone/stiffness) and dystonia (involuntary muscle contractions resulting in abnormal movement or posture), but many affected individuals are asymptomatic. Seizures and coma (encephalopathy) are rare. GA1 also causes secondary carnitine deficiency because 3-hydroxyglutaric acid, like other organic acids, is detoxified by carnitine. Glutaconic acid's neurotoxicity is thought to be partially caused by an excitotoxic mechanism in which glutaconic acid overactivates N-methyl-D-aspartate (NMDA) receptors. Accumulating trans-glutaconic (TG) acids have been proposed to be involved in the development of the striatal degeneration seen in children with glutaric acidemia type I via an excitotoxic mechanism.
trans-Glutaconic acidChEBI
(e)-Glutaconic acidGenerator
(e)-2-Pentenedioic acidHMDB
1,3-Propenedicarboxylic acidHMDB
1-Propene-1,3-dicarboxylic acidHMDB
Pentenedioic acidHMDB
Glutaconic acid, (e)-isomerHMDB
2-Pentenedioic acidHMDB
Glutaconic acidHMDB
Chemical FormulaC5H6O4
Average Molecular Weight130.0987
Monoisotopic Molecular Weight130.02660868
IUPAC Name(2E)-pent-2-enedioic acid
Traditional Nameglutaconic acid
CAS Registry Number1724-02-3
InChI Identifier
Chemical Taxonomy
Description belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic acids and derivatives
Alternative Parents
  • Fatty acyl
  • Fatty acid
  • Unsaturated fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Physiological effect

Health effect:



Biological location:


Naturally occurring process:


Indirect biological role:

Industrial application:

Biological role:

Physical Properties
Experimental Properties
Melting Point133 - 135 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility17.6 g/LALOGPS
pKa (Strongest Acidic)3.69ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity29.23 m³·mol⁻¹ChemAxon
Polarizability11.36 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001r-9100000000-42f59aea4f8e6bb8ab57Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-05g0-8920000000-733f5a9f839df29bff69Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-000i-9000000000-6fb6cc9ab31410b273f1Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-000f-9000000000-b9c27f4002ce9f12f916Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-000f-9100000000-d627e3ddfe858b1b331dSpectrum
LC-MS/MSLC-MS/MS Spectrum - QTOF 7V, positivesplash10-000i-9100000000-ab58b5793b44b9bf9179Spectrum
LC-MS/MSLC-MS/MS Spectrum - QTOF 10V, positivesplash10-00ds-9100000000-d837a612ccd5753681feSpectrum
LC-MS/MSLC-MS/MS Spectrum - QTOF 15V, positivesplash10-00ds-9000000000-297bd6690b68056be798Spectrum
LC-MS/MSLC-MS/MS Spectrum - QTOF 17V, positivesplash10-006t-9000000000-fcf4434d16f307cff090Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 0V, positivesplash10-03di-2900000000-2e649f3de9cd6e527eb8Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 0V, positivesplash10-03di-4900000000-49e2416d6fb162ad2653Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 0V, positivesplash10-03dr-6900000000-de40796dc1578c547169Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 0V, positivesplash10-01p9-9700000000-5d5dcebba638d34e8facSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, positivesplash10-01p9-9400000000-eb38aa127f9847501c88Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, positivesplash10-000i-9300000000-cd256964e84b2c9e7330Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, positivesplash10-000i-9100000000-ec0b1e5f63f16ae5f6baSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, positivesplash10-000i-9100000000-225d56adbb31a91aea12Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, positivesplash10-000i-9100000000-f7162d7b901bcae643baSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, positivesplash10-000i-9000000000-fa9f8d6b587fca352dd5Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, positivesplash10-000i-9000000000-77c952cabb0213b55f1eSpectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 3V, positivesplash10-0079-9000000000-e0422714c30ca69f9b7eSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03e9-3900000000-f982ee0bd6ebcaf33bbeSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01q0-9500000000-90dd8c522781e5da5ebbSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00n0-9000000000-e56400244a361d348a1fSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-1900000000-ad86855e24f296413178Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01t9-4900000000-30e4a1adead132f7de92Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-052f-9000000000-47a3aa8cf8913cc4d70eSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Urine
Tissue LocationsNot Available
Normal Concentrations
UrineDetected and Quantified0-2 umol/mmol creatinineNewborn (0-30 days old)Both
UrineDetected and Quantified3.1 (1.2-3.1) umol/mmol creatinineAdult (>18 years old)Both
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNot Available details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
    • BC Children's Hos...
Abnormal Concentrations
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothGlutaric aciduria type 1
    • MetaGene: Metabol...
UrineDetected and Quantified180.0 (0.00-360.0) umol/mmol creatinineChildren (1-13 years old)BothGlutaric aciduria I
    • MetaGene: Metabol...
Associated Disorders and Diseases
Disease References
Glutaric aciduria I
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: METAGENE consortium.
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022146
KNApSAcK IDNot Available
Chemspider ID4444138
KEGG Compound IDC02214
BiGG IDNot Available
Wikipedia LinkGlutaconic_acid
PubChem Compound5280498
PDB IDNot Available
ChEBI ID15670
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000203
Synthesis ReferenceSchwarz, Siegfried; Bohn, Helmut; Schmidt, Dieter; Kulpa, Manfred; Spakowski, Horst; Becker, Manfred. Glutaconic acid. Ger. (East) (1967), 2 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Podebrad F, Heil M, Beck T, Mosandl A, Sewell AC, Bohles H: Stereodifferentiation of 3-hydroxyisobutyric- and 3-aminoisobutyric acid in human urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry. Clin Chim Acta. 2000 Feb 25;292(1-2):93-105. [PubMed:10686279 ]
  2. Osaka H, Kimura S, Nezu A, Yamazaki S, Saitoh K, Yamaguchi S: Chronic subdural hematoma, as an initial manifestation of glutaric aciduria type-1. Brain Dev. 1993 Mar-Apr;15(2):125-7. [PubMed:8214331 ]
  3. Wang X, Wang X, Xie G, Zhou M, Yu H, Lin Y, Du G, Luo G, Jia W, Liu P: Urinary metabolite variation is associated with pathological progression of the post-hepatitis B cirrhosis patients. J Proteome Res. 2012 Jul 6;11(7):3838-47. doi: 10.1021/pr300337s. Epub 2012 Jun 7. [PubMed:22624806 ]