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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:44:19 UTC
HMDB IDHMDB0000620
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 1.3.99.7, 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.
Structure
Data?1563860659
Synonyms
ValueSource
(e)-GlutaconateChEBI
trans-GlutaconateChEBI
trans-Glutaconic acidChEBI
(e)-Glutaconic acidGenerator
GlutaconateGenerator
(e)-2-PentenedioateHMDB
(e)-2-Pentenedioic acidHMDB
1,3-PropenedicarboxylateHMDB
1,3-Propenedicarboxylic acidHMDB
1-Propene-1,3-dicarboxylateHMDB
1-Propene-1,3-dicarboxylic acidHMDB
PentenedioateHMDB
Pentenedioic acidHMDB
Glutaconic acid, (e)-isomerHMDB
2-PentendioateHMDB
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
SMILES
OC(=O)C\C=C\C(O)=O
InChI Identifier
InChI=1S/C5H6O4/c6-4(7)2-1-3-5(8)9/h1-2H,3H2,(H,6,7)(H,8,9)/b2-1+
InChI KeyXVOUMQNXTGKGMA-OWOJBTEDSA-N
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
Substituents
  • 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
Ontology
Physiological effect

Health effect:

Disposition

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point133 - 135 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility17.6 g/LALOGPS
logP0.05ALOGPS
logP0.044ChemAxon
logS-0.87ALOGPS
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
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3930000000-d1b6e04bed9f3de84afaJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-3910000000-02c8fdec1f78bd5117e2JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001r-9100000000-42f59aea4f8e6bb8ab57JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-05g0-8920000000-733f5a9f839df29bff69JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-000i-9000000000-6fb6cc9ab31410b273f1JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-000f-9000000000-b9c27f4002ce9f12f916JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-000f-9100000000-d627e3ddfe858b1b331dJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03e9-3900000000-f982ee0bd6ebcaf33bbeJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01q0-9500000000-90dd8c522781e5da5ebbJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00n0-9000000000-e56400244a361d348a1fJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-1900000000-ad86855e24f296413178JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01t9-4900000000-30e4a1adead132f7de92JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-052f-9000000000-47a3aa8cf8913cc4d70eJSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified3.1 (1.2-3.1) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not Quantified Adult (>18 years old)BothNot Available details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothGlutaric aciduria type 1
    • MetaGene: Metabol...
details
UrineDetected and Quantified180.0 (0.00-360.0) umol/mmol creatinineChildren (1-13 years old)BothGlutaric aciduria I
    • MetaGene: Metabol...
details
Associated Disorders and Diseases
Disease References
Glutaric aciduria I
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022146
KNApSAcK IDNot Available
Chemspider ID4444138
KEGG Compound IDC02214
BioCyc IDGLUTACONATE
BiGG IDNot Available
Wikipedia LinkGlutaconic_acid
METLIN ID5593
PubChem Compound5280498
PDB IDNot Available
ChEBI ID15670
References
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 ]