Record Information |
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Version | 5.0 |
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Status | Detected and Quantified |
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Creation Date | 2005-11-16 15:48:42 UTC |
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Update Date | 2023-02-21 17:14:59 UTC |
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HMDB ID | HMDB0000620 |
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Secondary Accession Numbers | - HMDB0001657
- HMDB0061185
- HMDB00620
- HMDB01657
- HMDB61185
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Metabolite Identification |
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Common Name | Glutaconic acid |
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Description | Glutaconic 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. |
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Structure | InChI=1S/C5H6O4/c6-4(7)2-1-3-5(8)9/h1-2H,3H2,(H,6,7)(H,8,9)/b2-1+ |
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Synonyms | Value | Source |
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(e)-Glutaconate | ChEBI | trans-Glutaconate | ChEBI | trans-Glutaconic acid | ChEBI | (e)-Glutaconic acid | Generator | Glutaconate | Generator | (e)-2-Pentenedioate | HMDB | (e)-2-Pentenedioic acid | HMDB | 1,3-Propenedicarboxylate | HMDB | 1,3-Propenedicarboxylic acid | HMDB | 1-Propene-1,3-dicarboxylate | HMDB | 1-Propene-1,3-dicarboxylic acid | HMDB | Pentenedioate | HMDB | Pentenedioic acid | HMDB | Glutaconic acid, (e)-isomer | HMDB | 2-Pentendioate | HMDB | 2-Pentenedioic acid | HMDB | Glutaconic acid | HMDB |
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Chemical Formula | C5H6O4 |
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Average Molecular Weight | 130.0987 |
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Monoisotopic Molecular Weight | 130.02660868 |
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IUPAC Name | (2E)-pent-2-enedioic acid |
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Traditional Name | glutaconic acid |
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CAS Registry Number | 1724-02-3 |
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SMILES | OC(=O)C\C=C\C(O)=O |
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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+ |
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InChI Key | XVOUMQNXTGKGMA-OWOJBTEDSA-N |
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Chemical Taxonomy |
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Description | Belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups. |
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Kingdom | Organic compounds |
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Super Class | Organic acids and derivatives |
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Class | Carboxylic acids and derivatives |
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Sub Class | Dicarboxylic acids and derivatives |
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Direct Parent | Dicarboxylic acids and derivatives |
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Alternative Parents | |
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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
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | |
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Ontology |
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Physiological effect | |
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Disposition | |
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Process | |
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Role | |
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Physical Properties |
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State | Solid |
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Experimental Molecular Properties | Property | Value | Reference |
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Melting Point | 133 - 135 °C | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | Not Available | Not Available | LogP | Not Available | Not Available |
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Experimental Chromatographic Properties | Not Available |
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Predicted Molecular Properties | |
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Predicted Chromatographic Properties | Predicted Collision Cross SectionsPredicted Kovats Retention IndicesUnderivatizedDerivatizedDerivative Name / Structure | SMILES | Kovats RI Value | Column Type | Reference |
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Glutaconic acid,1TMS,isomer #1 | C[Si](C)(C)OC(=O)C/C=C/C(=O)O | 1370.2 | Semi standard non polar | 33892256 | Glutaconic acid,1TMS,isomer #2 | C[Si](C)(C)OC(=O)/C=C/CC(=O)O | 1376.1 | Semi standard non polar | 33892256 | Glutaconic acid,2TMS,isomer #1 | C[Si](C)(C)OC(=O)/C=C/CC(=O)O[Si](C)(C)C | 1427.0 | Semi standard non polar | 33892256 | Glutaconic acid,1TBDMS,isomer #1 | CC(C)(C)[Si](C)(C)OC(=O)C/C=C/C(=O)O | 1624.9 | Semi standard non polar | 33892256 | Glutaconic acid,1TBDMS,isomer #2 | CC(C)(C)[Si](C)(C)OC(=O)/C=C/CC(=O)O | 1636.6 | Semi standard non polar | 33892256 | Glutaconic acid,2TBDMS,isomer #1 | CC(C)(C)[Si](C)(C)OC(=O)/C=C/CC(=O)O[Si](C)(C)C(C)(C)C | 1882.8 | Semi standard non polar | 33892256 |
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| GC-MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3930000000-d1b6e04bed9f3de84afa | 2017-09-12 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3910000000-02c8fdec1f78bd5117e2 | 2017-09-12 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3930000000-d1b6e04bed9f3de84afa | 2018-05-18 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3910000000-02c8fdec1f78bd5117e2 | 2018-05-18 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3930000000-d1b6e04bed9f3de84afa | 2018-05-18 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3910000000-02c8fdec1f78bd5117e2 | 2018-05-18 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3930000000-d1b6e04bed9f3de84afa | 2018-05-18 | HMDB team, MONA, MassBank | View Spectrum | Experimental GC-MS | GC-MS Spectrum - Glutaconic acid GC-EI-TOF (Non-derivatized) | splash10-0002-3910000000-02c8fdec1f78bd5117e2 | 2018-05-18 | HMDB team, MONA, MassBank | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (Non-derivatized) - 70eV, Positive | splash10-001r-9100000000-42f59aea4f8e6bb8ab57 | 2017-09-01 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (2 TMS) - 70eV, Positive | splash10-05g0-8920000000-733f5a9f839df29bff69 | 2017-10-06 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (Non-derivatized) - 70eV, Positive | Not Available | 2021-10-12 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (TMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (TMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (TBDMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (TBDMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Glutaconic acid GC-MS (TBDMS_2_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum |
MS/MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Quattro_QQQ 10V, Negative-QTOF (Annotated) | splash10-000i-9000000000-6fb6cc9ab31410b273f1 | 2012-07-24 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Quattro_QQQ 25V, Negative-QTOF (Annotated) | splash10-000f-9000000000-b9c27f4002ce9f12f916 | 2012-07-24 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Quattro_QQQ 40V, Negative-QTOF (Annotated) | splash10-000f-9100000000-d627e3ddfe858b1b331d | 2012-07-24 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid QTOF 7V, positive-QTOF | splash10-000i-9100000000-ab58b5793b44b9bf9179 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid QTOF 10V, positive-QTOF | splash10-00ds-9100000000-d837a612ccd5753681fe | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid QTOF 15V, positive-QTOF | splash10-00ds-9000000000-297bd6690b68056be798 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid QTOF 17V, positive-QTOF | splash10-006t-9000000000-fcf4434d16f307cff090 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 0V, positive-QTOF | splash10-03di-2900000000-2e649f3de9cd6e527eb8 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 0V, positive-QTOF | splash10-03di-4900000000-49e2416d6fb162ad2653 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 0V, positive-QTOF | splash10-03dr-6900000000-de40796dc1578c547169 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 0V, positive-QTOF | splash10-01p9-9700000000-5d5dcebba638d34e8fac | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 1V, positive-QTOF | splash10-01p9-9400000000-eb38aa127f9847501c88 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 1V, positive-QTOF | splash10-000i-9300000000-cd256964e84b2c9e7330 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 1V, positive-QTOF | splash10-000i-9100000000-ec0b1e5f63f16ae5f6ba | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 1V, positive-QTOF | splash10-000i-9100000000-225d56adbb31a91aea12 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 2V, positive-QTOF | splash10-000i-9100000000-f7162d7b901bcae643ba | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 2V, positive-QTOF | splash10-000i-9000000000-fa9f8d6b587fca352dd5 | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 2V, positive-QTOF | splash10-000i-9000000000-77c952cabb0213b55f1e | 2020-07-22 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Glutaconic acid Orbitrap 3V, positive-QTOF | splash10-0079-9000000000-e0422714c30ca69f9b7e | 2020-07-22 | HMDB team, MONA | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Glutaconic acid 10V, Positive-QTOF | splash10-03e9-3900000000-f982ee0bd6ebcaf33bbe | 2017-09-01 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Glutaconic acid 20V, Positive-QTOF | splash10-01q0-9500000000-90dd8c522781e5da5ebb | 2017-09-01 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Glutaconic acid 40V, Positive-QTOF | splash10-00n0-9000000000-e56400244a361d348a1f | 2017-09-01 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Glutaconic acid 10V, Negative-QTOF | splash10-004i-1900000000-ad86855e24f296413178 | 2017-09-01 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Glutaconic acid 20V, Negative-QTOF | splash10-01t9-4900000000-30e4a1adead132f7de92 | 2017-09-01 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Glutaconic acid 40V, Negative-QTOF | splash10-052f-9000000000-47a3aa8cf8913cc4d70e | 2017-09-01 | Wishart Lab | View Spectrum |
NMR SpectraSpectrum Type | Description | Deposition Date | Source | View |
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Experimental 1D NMR | 1H NMR Spectrum (1D, 500 MHz, H2O, experimental) | 2012-12-04 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 100 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 100 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 1000 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 1000 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 200 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 200 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 300 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 300 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 400 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 400 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 500 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 500 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 600 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 600 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 700 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 700 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 800 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 800 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 900 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 900 MHz, D2O, predicted) | 2021-09-24 | Wishart Lab | View Spectrum | Experimental 2D NMR | [1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental) | 2012-12-05 | Wishart Lab | View Spectrum |
IR SpectraSpectrum Type | Description | Deposition Date | Source | View |
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Predicted IR Spectrum | IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-) | 2023-02-03 | FELIX lab | View Spectrum | Predicted IR Spectrum | IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+) | 2023-02-03 | FELIX lab | View Spectrum | Predicted IR Spectrum | IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+) | 2023-02-03 | FELIX lab | View Spectrum |
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