Record Information |
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Version | 2.0 |
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Creation Date | 2014-08-29 06:11:12 UTC |
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Update Date | 2018-03-21 17:46:15 UTC |
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Accession Number | T3D4270 |
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Identification |
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Common Name | Pyroglutamic acid |
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Class | Small Molecule |
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Description | Pyroglutamic acid (5-oxoproline) is a cyclized derivative of L-glutamic acid. It is an uncommon amino acid derivative in which the free amino group of glutamic acid cyclizes to form a lactam. It is formed nonenzymatically from glutamate, glutamine, and gamma-glutamylated peptides, but it can also be produced by the action of gamma-glutamylcyclotransferase on an L-amino acid. Elevated blood levels may be associated with problems of glutamine or glutathione metabolism. This compound is found in substantial amounts in brain tissue and other tissues in bound form, especially skin. It is also present in plant tissues. It is sold, over the counter, as a "smart drug" for improving blood circulation in the brain. Pyroglutamate in the urine is a biomarker for the consumption of cheese. When present in sufficiently high levels, pyroglutamic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of pyroglutamic acid are associated with at least five inborn errors of metabolism including 5-oxoprolinuria, 5-oxoprolinase deficiency, glutathione synthetase deficiency, hawkinsinuria, and propionic acidemia. Pyroglutamic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. |
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Compound Type | - Animal Toxin
- Food Toxin
- Metabolite
- Natural Compound
- Organic Compound
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Chemical Structure | |
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Synonyms | Synonym | (-)-2-Pyrrolidone-5-carboxylate | (-)-2-Pyrrolidone-5-carboxylic acid | (-)-Pyroglutamate | (-)-Pyroglutamic acid | (5S)-2-Oxopyrrolidine-5-carboxylate | (5S)-2-Oxopyrrolidine-5-carboxylic acid | (S)-(-)-2-Pyrrolidone-5-carboxylate | (S)-(-)-2-Pyrrolidone-5-carboxylic acid | (S)-(-)-g-Butyrolactam-g-carboxylate | (S)-(-)-g-Butyrolactam-g-carboxylic acid | (S)-(-)-gamma-Butyrolactam-gamma-carboxylate | (S)-(-)-gamma-Butyrolactam-gamma-carboxylic acid | (S)-2-Pyrrolidone-5-carboxylate | (S)-2-Pyrrolidone-5-carboxylic acid | (S)-5-Oxo-2-pyrrolidinecarboxylate | (S)-5-Oxo-2-pyrrolidinecarboxylic acid | (S)-Pyroglutamate | (S)-Pyroglutamic acid | 2-L-Pyrrolidone-5-carboxylate | 2-L-Pyrrolidone-5-carboxylic acid | 2-Oxopyrrolidine-5(S)-carboxylate | 2-Oxopyrrolidine-5(S)-carboxylic acid | 2-Pyrrolidinone-5-carboxylate | 2-Pyrrolidinone-5-carboxylic acid | 5-Carboxy-2-pyrrolidinone | 5-L-Oxoproline | 5-Oxo-L-proline | 5-Oxoproline | 5-Pyrrolidinone-2-carboxylate | 5-Pyrrolidinone-2-carboxylic acid | Ajidew A 100 | Glutimate | Glutimic acid | Glutiminate | Glutiminic acid | L-2-Pyrrolidone-5-carboxylate | L-2-Pyrrolidone-5-carboxylic acid | L-5-Carboxy-2-pyrrolidinone | L-5-Oxo-2-pyrrolidinecarboxylate | L-5-Oxo-2-pyrrolidinecarboxylic acid | L-5-Oxoproline | L-Glutamic acid g-lactam | L-Glutimate | L-Glutimic acid | L-Glutiminate | L-Glutiminic acid | L-Pyroglutamate | L-Pyroglutamic acid | L-Pyrrolidinonecarboxylate | L-Pyrrolidinonecarboxylic acid | L-Pyrrolidonecarboxylate | L-Pyrrolidonecarboxylic acid | Oxoproline | Oxopyrrolidinecarboxylate | Oxopyrrolidinecarboxylic acid | Pidolate | Pidolic acid | Pidolidone | Pyroglutamate | Pyrrolidinonecarboxylate | Pyrrolidinonecarboxylic acid | Pyrrolidone-5-carboxylate | Pyrrolidone-5-carboxylic acid | Pyrrolidonecarboxylic acid |
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Chemical Formula | C5H7NO3 |
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Average Molecular Mass | 129.114 g/mol |
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Monoisotopic Mass | 129.043 g/mol |
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CAS Registry Number | 98-79-3 |
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IUPAC Name | (2S)-5-oxopyrrolidine-2-carboxylic acid |
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Traditional Name | pyroglutamic acid |
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SMILES | [H][C@]1(CCC(O)=N1)C(O)=O |
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InChI Identifier | InChI=1S/C5H7NO3/c7-4-2-1-3(6-4)5(8)9/h3H,1-2H2,(H,6,7)(H,8,9)/t3-/m0/s1 |
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InChI Key | InChIKey=ODHCTXKNWHHXJC-VKHMYHEASA-N |
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Chemical Taxonomy |
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Description | belongs to the class of organic compounds known as alpha amino acids and derivatives. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon), or a derivative thereof. |
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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 | Amino acids, peptides, and analogues |
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Direct Parent | Alpha amino acids and derivatives |
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Alternative Parents | |
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Substituents | - Alpha-amino acid or derivatives
- Pyrroline carboxylic acid
- Pyrroline carboxylic acid or derivatives
- Pyrroline
- Cyclic carboximidic acid
- Lactim
- Carboxylic acid
- Monocarboxylic acid or derivatives
- Propargyl-type 1,3-dipolar organic compound
- Organic 1,3-dipolar compound
- Azacycle
- Organoheterocyclic compound
- Organopnictogen compound
- Organic oxygen compound
- Organooxygen compound
- Organic oxide
- Hydrocarbon derivative
- Carbonyl group
- Organic nitrogen compound
- Organonitrogen compound
- Aliphatic heteromonocyclic compound
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Molecular Framework | Aliphatic heteromonocyclic compounds |
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External Descriptors | |
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Biological Properties |
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Status | Detected and Not Quantified |
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Origin | Endogenous |
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Cellular Locations | |
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Biofluid Locations | Not Available |
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Tissue Locations | |
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Pathways | |
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Applications | Not Available |
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Biological Roles | Not Available |
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Chemical Roles | Not Available |
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Physical Properties |
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State | Solid |
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Appearance | White powder. |
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Experimental Properties | Property | Value |
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Melting Point | 158°C (316.4°F) | Boiling Point | Not Available | Solubility | 476.0 mg/mL at 13°C | LogP | Not Available |
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Predicted Properties | |
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Spectra |
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Spectra | Spectrum Type | Description | Splash Key | Deposition Date | View |
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GC-MS | GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS) | splash10-0ab9-8900000000-f79dc90370ba38f587c9 | 2014-06-16 | View Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-0ab9-8900000000-f79dc90370ba38f587c9 | 2017-09-12 | View Spectrum | GC-MS | GC-MS Spectrum - GC-EI-TOF (Non-derivatized) | splash10-0a4i-0900000000-90fb43273551aeb9b2c4 | 2017-09-12 | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | splash10-0a6r-9000000000-130a8f31f82e83c4be07 | 2016-09-22 | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive | splash10-05fr-9200000000-d69b52257404ab658d5b | 2017-10-06 | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | Not Available | 2021-10-12 | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated) | splash10-001i-9500000000-ebc64308ec5d5bdb303e | 2012-07-24 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated) | splash10-053r-9000000000-7377cb17491942e9589c | 2012-07-24 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated) | splash10-053u-9000000000-fcab1396867356ebd6ae | 2012-07-24 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative | splash10-004i-0900000000-5b0c6536e1b3217b8544 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative | splash10-004i-0900000000-c30ac0bd264c8007ef92 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative | splash10-004i-5900000000-ea3a164653e4235716ae | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative | splash10-0f89-9000000000-f6620738e68f990d0594 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative | splash10-0udi-9000000000-7937bee2e9a6d6b29cbd | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative | splash10-004i-0900000000-f20401903b234914b936 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative | splash10-004i-0900000000-9446bb65e0edd72cfd59 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative | splash10-0059-7900000000-74eccdeb9f0d5fd17614 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative | splash10-0f8a-9000000000-8786a9cd5e488192f34d | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative | splash10-0f6t-9000000000-ebcc1ac4acd525218e80 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive | splash10-01q9-2900000000-754ae9b699ec1b22cd76 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive | splash10-001i-9300000000-eabb8c4dc0d1111e0431 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive | splash10-00lr-9100000000-1dd17702aee7e5bce618 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive | splash10-067i-9000000000-c9669794d3a8746be498 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive | splash10-014i-9000000000-9e103abb0a6ed890051e | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive | splash10-03e9-3900000000-da8cf252285c1d616586 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive | splash10-01q9-9400000000-96a7fe5a81188c49d1ba | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive | splash10-014i-9000000000-356215339a43217dea66 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive | splash10-02vl-9000000000-ed47ec6e675eb338da19 | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive | splash10-014i-9000000000-f647da344adbdf7bfb1b | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive | splash10-001i-9200000000-0bddc68d58c6fb981d1a | 2012-08-31 | View Spectrum | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative | splash10-004i-0900000000-c70c79fa828bbf137ebc | 2012-08-31 | View Spectrum | MS | Mass Spectrum (Electron Ionization) | splash10-001i-9000000000-6c87253da642bb4800df | 2019-05-16 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 600 MHz, H2O, experimental) | Not Available | 2012-12-04 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 100 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 100 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 1000 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 1000 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 200 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 200 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 300 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 300 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 400 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 400 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 500 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 500 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 600 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 600 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 700 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 700 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 800 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 800 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 13C NMR Spectrum (1D, 900 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 1D NMR | 1H NMR Spectrum (1D, 900 MHz, D2O, predicted) | Not Available | 2021-09-29 | View Spectrum | 2D NMR | [1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental) | Not Available | 2012-12-05 | View Spectrum |
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Toxicity Profile |
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Route of Exposure | Not Available |
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Mechanism of Toxicity | 5-Oxoprolinuria develops in moderate to severe cases of glutathione synthetase deficiency. The deficiency in glutathione synthetase leads to the accumulation of γ-glutamylcysteine, which is converted into 5-oxoproline by the action of γ-glutamyl cyclotransferase. The excessive formation of 5-oxoproline exceeds the capacity of 5-oxoprolinase, leading to accumulation of 5-oxoproline in body fluids causing metabolic acidosis and 5-oxoprolinuria. 5-Oxoproline accumulation is thought to be the cause of metabolic acidosis in Hawkinsinuria. 5-Oxoprolinase deficiency also leads to decreased conversion of 5-oxoproline to glutamate, resulting in elevated levels of 5-oxoproline in body fluids. 5-Oxoprolinuria has also been described in patients with urea cycle defects, such as ornithine transcarbamoylase deficiency or homocystinuria. In nephropathic cystinosis 5-oxoprolinuria may occur because of secondary impairment of the γ-glutamyl cycle resulting from decreased availability of free cysteine and can be corrected through cysteamine therapy. Transient 5-oxoprolinuria of unknown cause has been reported in very preterm infants. Limited availability of glycine in malnutrition and pregnancy as well as increased turnover of collagen, fibrinogen and other proteins containing considerable amounts of 5-oxoproline in patients with severe burns or Stevens-Johnson syndrome may lead to 5-oxoprolinuria. In addition, certain drugs, such as paracetamol, vigabatrin or some antibiotics (flucloxacillin, netimicin), are known to induce 5-oxoprolinuria, probably through interaction with the γ-glutamyl cycle. Particular infant formulas and tomato juice may contain modified proteins with increased content of 5-oxoproline. (15) |
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Metabolism | 5-Oxoproline is part of the glutathione metabolism pathway. Degradation of glutathione is initiated by γ-glutamyl transpeptidase, which catalyses the transfer of its γ-glutamyl-group to acceptors. The γ-glutamyl residues are substrates of the γ-glutamyl-cyclotransferase, which converts them to 5-oxoproline and the corresponding amino acids. Conversion of 5-oxoproline to glutamate is catalysed by 5-oxoprolinase. (15) |
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Toxicity Values | Not Available |
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Lethal Dose | Not Available |
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Carcinogenicity (IARC Classification) | No indication of carcinogenicity to humans (not listed by IARC). |
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Uses/Sources | This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. |
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Minimum Risk Level | Not Available |
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Health Effects | Chronically high levels of pyroglutamic acid are associated with at least 5 inborn errors of metabolism including: 5-Oxoprolinuria, 5-oxoprolinase deficiency, Glutathione Synthetase Deficiency, Hawkinsinuria and Propionic acidemia. |
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Symptoms | Patients with the moderate variant of glutathione synthetase deficiency usually present during the neonatal period with severe and chronic metabolic acidosis, mild to moderate haemolytic anaemia, jaundice and 5-oxoprolinuria. After the neonatal period, the condition usually stabilises, but patients may become critically ill during infections owing to pronounced acidosis and electrolyte imbalances. Several patients have died during such episodes. In addition to the symptoms mentioned above, patients with severe GS deficiency develop progressive CNS symptoms, e.g. mental retardation, seizures, spasticity, ataxia and intention tremor. In addition, some patients suffer from recurrent severe bacterial infections, which is probably due to defective granulocyte function. Hawkinsinuria is characterised by failure to thrive and metabolic acidosis in infancy. After the 1st year of life the condition appears to be asymptomatic. Early weaning from breastfeeding seems to precipitate the disease; the condition may be asymptomatic in breastfed infants. 5-Oxoprolinase Deficiency: Up to now, eight patients in six different families have been described. The clinical symptoms are inconstant and very heterogeneous, including renal stone formation, enterocolitis, neonatal hypoglycaemia, microcytic anaemia, microcephaly and mental retardation. It remains to be established wheter symptoms in identified patients are merely a coincidence. (15) |
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Treatment | Glutathione Synthetase Deficiency: The clinical management of GS deficient patients is aimed at correction of acidosis, prevention of haemolytic crises and support of endogenous defence against reactive oxygen species (ROS). In the neonatal period, correction of metabolic acidosis, electrolyte imbalances, treatment of anaemia and excessive hyperbilirubinaemia are of crucial importance. Correction of acidosis can be reached through bicarbonate, citrate or tris-hydroxymethyl aminomethane (THAM). Doses of up to 10 mmol/kg/day, or even higher in episodes of acute infections, may be required. Repeated blood transfusions may be necessary in patients with massive haemolysis. Drugs and foods known to precipitate haemolytic crises in patients with glucose- 6-phosphatase dehydrogenase deficiency should be avoided. Successful treatment with erythropoietin has been reported in one patient. Early supplementation with vitamin E and vitamin C are thought to replenish the lack of GSH as a scavenger of free radicals. Recommended doses are 10 mg/kg/day for vitamin E and 100 mg/kg/day for vitamin C. A longterm follow-up study of 28 patients suggested that early supplementation with both vitamins may prevent CNS damage and improve the long-term clinical outcome in GS-deficient patients. The value of N-acetylcysteine, which is known to protect cells from oxidative stress in vitro, in the treatment of GS deficiency is controversial. It was suggested that the low intracellular GSH concentrations and cysteine availabilty might be increased by N-acetylcysteine. However, supplementation with N-acetylcysteine should not be recommended, because it was shown at least in cultured fibroblasts that patients with GS deficiency accumulate cysteine, which is known to be neurotoxic in excessive amounts. A therapeutic trial with orally administered GSH showed no lasting benefit in two patients with GS deficiency. GSH esters, lipid-soluble preparations which are easily transported into cells where they are converted into GSH, have been tried in animal models of GSH deficiency and in two patients with GS deficiency. However, associated toxic effects due to production of alcohols as a by-product during hydrolysis to release GSH make them of limited use. In vitro studies have shown that addition of S-acetylglutathione to the medium of cultured fibroblasts from patients with GS deficiency normalised intracellular GSH content. Owing to the rarity of the disease and the heterogeneity of the clinical condition the prognosis for individual patients is difficult to predict. Early diagnosis, correction of acidosis and early supplementation with vitamin E and vitamin C appear to be the most important factors regarding the survival and the long-term outcome. Hawkinsinuria: Symptoms in infancy respond to a return to breastfeeding or a diet restricted in tyrosine and phenylalanine along with vitamin C supplementation. The condition is asymptomatic after the 1st year of life, and affected infants are reported to have developed normally. 5-Oxoprolinase Deficiency: No specific treatment has been proposed or tried. (15) |
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Normal Concentrations |
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| Not Available |
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Abnormal Concentrations |
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| Not Available |
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External Links |
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DrugBank ID | DB03088 |
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HMDB ID | HMDB00267 |
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PubChem Compound ID | 7405 |
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ChEMBL ID | CHEMBL397976 |
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ChemSpider ID | 7127 |
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KEGG ID | C01879 |
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UniProt ID | Not Available |
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OMIM ID | |
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ChEBI ID | 18183 |
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BioCyc ID | CPD-589 |
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CTD ID | Not Available |
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Stitch ID | Not Available |
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PDB ID | PCA |
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ACToR ID | Not Available |
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Wikipedia Link | Pyroglutamic acid |
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References |
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Synthesis Reference | John G. Black, Ian R. Scott, “Pyroglutamic acid esters, their synthesis and use in topical products.” U.S. Patent US4774255, issued December, 1974. |
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MSDS | Link |
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General References | - Manning NJ, Davies NP, Olpin SE, Carpenter KH, Smith MF, Pollitt RJ, Duncan SL, Larsson A, Carlsson B: Prenatal diagnosis of glutathione synthase deficiency. Prenat Diagn. 1994 Jun;14(6):475-8. [7937585 ]
- Caspers PJ, Lucassen GW, Carter EA, Bruining HA, Puppels GJ: In vivo confocal Raman microspectroscopy of the skin: noninvasive determination of molecular concentration profiles. J Invest Dermatol. 2001 Mar;116(3):434-42. [11231318 ]
- Hussain Z, Lannigan R, Stoakes L: A new approach for presumptive identification of clinically important streptococci. Zentralbl Bakteriol Mikrobiol Hyg A. 1984 Oct;258(1):74-9. [6441390 ]
- 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. [8087979 ]
- Creer MH, Lau BW, Jones JD, Chan KM: Pyroglutamic acidemia in an adult patient. Clin Chem. 1989 Apr;35(4):684-6. [2702756 ]
- Hammond JW, Potter M, Truscott R, Wilcken B: gamma-Glutamylglutamine identified in plasma and cerebrospinal fluid from hyperammonaemic patients. Clin Chim Acta. 1990 Dec 24;194(2-3):173-83. [2093471 ]
- Uhlhaas S, Lange H: Striatal deficiency of L-pyroglutamic acid in Huntington's disease is accompanied by increased plasma levels. Brain Res. 1988 Aug 2;457(1):196-9. [2971422 ]
- Jellum E, Stokke O, Eldjarn L: Combined use of gas chromatography, mass spectrometry, and computer in diagnosis and studies of metabolic disorders. Clin Chem. 1972 Aug;18(8):800-9. [4557757 ]
- Croal BL, Glen AC, Kelly CJ, Logan RW: Transient 5-oxoprolinuria (pyroglutamic aciduria) with systemic acidosis in an adult receiving antibiotic therapy. Clin Chem. 1998 Feb;44(2):336-40. [9474033 ]
- Winslow JW, Shih A, Bourell JH, Weiss G, Reed B, Stults JT, Goldsmith LT: Human seminal relaxin is a product of the same gene as human luteal relaxin. Endocrinology. 1992 May;130(5):2660-8. [1572287 ]
- Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [8412012 ]
- Wevers RA, Engelke U, Heerschap A: High-resolution 1H-NMR spectroscopy of blood plasma for metabolic studies. Clin Chem. 1994 Jul;40(7 Pt 1):1245-50. [8013094 ]
- Erasmus E, Mienie LJ, de Vries WN, de Wet WJ, Carlsson B, Larsson A: Prenatal analysis in two suspected cases of glutathione synthetase deficiency. J Inherit Metab Dis. 1993;16(5):837-43. [8295398 ]
- 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. [19212411 ]
- Saudubray, Jean-Marie, Georges van den Berghe, and John H. Walter, eds. 2012. Inborn Metabolic Diseases: Diagnosis and Treatment. 5th edition. Berlin: Springer. [ISBN: 978-3-642-15719-6]
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Gene Regulation |
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Up-Regulated Genes | Not Available |
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Down-Regulated Genes | Not Available |
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