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Record Information |
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Version | 4.0 |
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Status | Detected but not Quantified |
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Creation Date | 2005-11-16 15:48:42 UTC |
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Update Date | 2020-09-14 22:01:26 UTC |
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HMDB ID | HMDB0001301 |
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Secondary Accession Numbers | - HMDB0002240
- HMDB01301
- HMDB02240
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Metabolite Identification |
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Common Name | 1-Pyrroline-5-carboxylic acid |
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Description | 1-Pyrroline-5-carboxylic acid (CAS: 2906-39-0) is an enamine or an imino acid that forms upon the spontaneous dehydration of L-glutamate gamma-semialdehyde in aqueous solutions. The stereoisomer (S)-1-pyrroline-5-carboxylate is an intermediate in glutamate metabolism, arginine degradation, and proline biosynthesis and degradation. It can also be converted into or be formed from three amino acids: L-glutamate, L-ornithine, and L-proline. In particular, it is synthesized via the oxidation of proline by pyrroline-5-carboxylate reductase 1 (PYCR1) (EC 1.5.1.2) or by proline dehydrogenase (PRODH) (EC 1.5.99.8). It is hydrolyzed into L-glutamate by delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) (EC 1.5.1.12). It is also one of the few metabolites that can act as a precursor to other metabolites of both the urea cycle and the tricarboxylic acid (TCA) cycle. Under certain conditions, pyrroline-5-carboxylate can act as a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of pyrroline-5-carboxylate are associated with at least five inborn errors of metabolism, including hyperprolinemia type I, hyperprolinemia type II, iminoglycinuria, prolinemia type II, and pyruvate carboxylase deficiency. Hyperprolinemia type II results in high levels of pyrroline-5-carboxylate. People with hyperprolinemia type II have signs and symptoms that vary in severity, but they are more likely than type I to have seizures or intellectual disability. Pyrroline-5-carboxylate is highly reactive and excess quantities have been shown to cause cell death and apoptosis (PMID: 15548746 ). |
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Structure | |
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Synonyms | Value | Source |
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1-Pyrroline-5-carboxylate | ChEBI, KEGG | L-1-Pyrroline-5-carboxylate | ChEBI, KEGG | 1-Pyrroline-5-carboxylic acid | Generator | (S)-1-Pyrroline-5-carboxylic acid | Generator | L-1-Pyrroline-5-carboxylic acid | Generator | delta-1-Pyrroline-5-carboxylate | MeSH | delta-1-Pyrroline-5-carboxylate, (+-)-isomer | MeSH | delta-1-Pyrroline-5-carboxylic acid | MeSH | delta-1-Pyrroline-5-carboxylate, 14C-labeled, (+-)-isomer | MeSH | delta(1)Pyrroline-5-carboxylate | MeSH | Pyrroline-5-carboxylate | MeSH | (2S)-3,4-Dihydro-2H-pyrrole-2-carboxylic acid | HMDB | (S)-1-Pyrroline-5-carboxylate | HMDB | 3,4-Dihydro-2H-pyrrole-2-carboxylic acid | HMDB | delta1-Pyrroline-5-carboxylic acid | HMDB | Δ1-Pyrroline-5-carboxylic acid | HMDB |
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Chemical Formula | C5H7NO2 |
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Average Molecular Weight | 113.1146 |
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Monoisotopic Molecular Weight | 113.047678473 |
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IUPAC Name | (2S)-3,4-dihydro-2H-pyrrole-2-carboxylic acid |
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Traditional Name | L-1-pyrroline-5-carboxylate |
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CAS Registry Number | 64199-88-8 |
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SMILES | OC(=O)[C@@H]1CCC=N1 |
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InChI Identifier | InChI=1S/C5H7NO2/c7-5(8)4-2-1-3-6-4/h3-4H,1-2H2,(H,7,8)/t4-/m0/s1 |
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InChI Key | DWAKNKKXGALPNW-BYPYZUCNSA-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
- Carboxylic acid
- Monocarboxylic acid or derivatives
- Propargyl-type 1,3-dipolar organic compound
- Organic 1,3-dipolar compound
- Organoheterocyclic compound
- Azacycle
- Organopnictogen compound
- Organic oxygen compound
- Organooxygen compound
- Organonitrogen compound
- Organic oxide
- Carbonyl group
- Imine
- Organic nitrogen compound
- Hydrocarbon derivative
- Aliphatic heteromonocyclic compound
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Molecular Framework | Aliphatic heteromonocyclic compounds |
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External Descriptors | |
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Ontology |
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Disposition | Source: Biological location: |
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Process | Naturally occurring process: |
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Role | Indirect biological role: |
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Physical Properties |
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State | Solid |
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Experimental Properties | Property | Value | Reference |
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Melting Point | Not Available | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | Not Available | Not Available | LogP | Not Available | Not Available |
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Predicted Properties | |
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Spectra |
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| Spectrum Type | Description | Splash Key | View |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-03dj-9700000000-f2bc2275638f96dc74ac | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-014i-9100000000-72dff6639c24448bec9c | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-014l-9000000000-28eb1358005b28459deb | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-03di-5900000000-0720aa91caee03b9364e | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-02t9-9400000000-cfcc87863f675fb12691 | Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-00kf-9000000000-a15b91c200295166bf9e | Spectrum |
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Biological Properties |
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Cellular Locations | |
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Biospecimen Locations | |
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Tissue Locations | |
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Pathways | |
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Normal Concentrations |
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Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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| Not Available |
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Associated Disorders and Diseases |
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Disease References | None |
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Associated OMIM IDs | None |
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External Links |
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DrugBank ID | Not Available |
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Phenol Explorer Compound ID | Not Available |
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FooDB ID | FDB030153 |
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KNApSAcK ID | C00007606 |
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Chemspider ID | 389153 |
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KEGG Compound ID | C03912 |
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BioCyc ID | L-DELTA1-PYRROLINE_5-CARBOXYLATE |
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BiGG ID | Not Available |
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Wikipedia Link | 1-Pyrroline-5-carboxylic acid |
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METLIN ID | Not Available |
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PubChem Compound | 440162 |
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PDB ID | Not Available |
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ChEBI ID | 371 |
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Food Biomarker Ontology | Not Available |
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VMH ID | Not Available |
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MarkerDB ID | |
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References |
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Synthesis Reference | Vogel, Henry J.; Davis, Bernard D. Glutamic g-semialdehyde and D1-pyrroline-5-carboxylic acid, intermediates in the biosynthesis of proline. Journal of the American Chemical Society (1952), 74 109-12. |
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Material Safety Data Sheet (MSDS) | Not Available |
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General References | - Simila S: Hydroxyproline metabolism in type II hyperprolinaemia. Ann Clin Biochem. 1979 Jul;16(4):177-81. [PubMed:533224 ]
- Mixson AJ, Phang JM: The uptake of pyrroline 5-carboxylate. Group translocation mediating the transfer of reducing-oxidizing potential. J Biol Chem. 1988 Aug 5;263(22):10720-4. [PubMed:3392037 ]
- Wakabayashi Y: Tissue-selective expression of enzymes of arginine synthesis. Curr Opin Clin Nutr Metab Care. 1998 Jul;1(4):335-9. [PubMed:10565370 ]
- Onenli-Mungan N, Yuksel B, Elkay M, Topaloglu AK, Baykal T, Ozer G: Type II hyperprolinemia: a case report. Turk J Pediatr. 2004 Apr-Jun;46(2):167-9. [PubMed:15214748 ]
- Fleming GA, Hagedorn CH, Granger AS, Phang JM: Pyrroline-5-carboxylate in human plasma. Metabolism. 1984 Aug;33(8):739-42. [PubMed:6748947 ]
- Humbertclaude V, Rivier F, Roubertie A, Echenne B, Bellet H, Vallat C, Morin D: Is hyperprolinemia type I actually a benign trait? Report of a case with severe neurologic involvement and vigabatrin intolerance. J Child Neurol. 2001 Aug;16(8):622-3. [PubMed:11510941 ]
- Deuschle K, Funck D, Forlani G, Stransky H, Biehl A, Leister D, van der Graaff E, Kunze R, Frommer WB: The role of [Delta]1-pyrroline-5-carboxylate dehydrogenase in proline degradation. Plant Cell. 2004 Dec;16(12):3413-25. doi: 10.1105/tpc.104.023622. Epub 2004 Nov 17. [PubMed:15548746 ]
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