Record Information |
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Version |
1.0 |
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Update Date |
1/22/2018 12:54:54 PM |
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Metabolite ID | PAMDB110829 |
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Identification |
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Name: |
L-arginino-succinate |
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Description: | Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the arginosuccinate lyase enzyme can lead to arginosuccinate lyase deficiency. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (arginosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema. |
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Structure |
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Synonyms: | -
N-(L-arginino)-succinate
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arginino-succinate
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Chemical Formula: |
C10H17N4O6
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Average Molecular Weight: |
289.27 |
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Monoisotopic Molecular
Weight: |
292.1382843954 |
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InChI Key: |
KDZOASGQNOPSCU-ZBHICJROSA-M |
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InChI: |
InChI=1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/p-1/t5-,6?/m0/s1 |
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CAS
number: |
2387-71-5 |
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IUPAC Name: | 2-(Nω-L-arginino)butanedioic acid |
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Traditional IUPAC Name: |
argininosuccinic acid |
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SMILES: | C(NC(=[N+])NC(CC(=O)[O-])C(=O)[O-])CCC(C(=O)[O-])[N+] |
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Chemical Taxonomy |
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Taxonomy Description | This compound belongs to the class of chemical entities known as aspartic acid and derivatives. These are compounds containing an aspartic acid or a derivative thereof resulting from reaction of aspartic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. |
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Kingdom |
Chemical entities |
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Super Class | Organic compounds |
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Class |
Organic acids and derivatives |
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Sub Class | Carboxylic acids and derivatives |
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Direct Parent |
Aspartic acid and derivatives |
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Alternative Parents |
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Substituents |
- Aspartic acid or derivatives
- Alpha-amino acid
- L-alpha-amino acid
- Tricarboxylic acid or derivatives
- Guanidine
- Amino acid
- Organic 1,3-dipolar compound
- Propargyl-type 1,3-dipolar organic compound
- Carboximidamide
- Carboxylic acid
- Hydrocarbon derivative
- Organopnictogen compound
- Organic oxygen compound
- Primary amine
- Organooxygen compound
- Organonitrogen compound
- Primary aliphatic amine
- Carbonyl group
- Amine
- Organic nitrogen compound
- Organic oxide
- Aliphatic acyclic compound
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Molecular Framework |
Aliphatic acyclic compounds |
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External Descriptors |
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Physical Properties |
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State: |
Solid |
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Charge: | 0 |
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Melting point: |
Not Available |
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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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Biological Properties |
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Cellular Locations: |
Not Available |
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Reactions: | |
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Pathways: |
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Spectra |
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Spectra: |
Spectrum Type | Description | Splash Key | |
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GC-MS | GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) | splash10-0007-0921100000-638b5887a29712423afd | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative | splash10-000i-0090000000-27e7b2225bca7ed0bfef | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative | splash10-00di-0390000000-b66871354ad66743ff5f | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative | splash10-001i-1910000000-777b6f2263b573f38bca | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative | splash10-001i-3900000000-26d6466903550c40fde6 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative | splash10-001r-8900000000-4e5455a5af85574ac9df | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive | splash10-0006-0090000000-79b7ca9043632dc877e1 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive | splash10-0006-0590000000-8fb4f4625f241d387e92 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive | splash10-06di-3910000000-55530a4477a3fde8c1c1 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive | splash10-00di-9200000000-413c3029964c0386047a | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive | splash10-00di-9000000000-6ffc3b2b5da5f69de80b | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-060r-0950000000-129ee81e1370707d975d | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-0a4i-0910000000-62431d9967ced6c96b28 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-03di-1980000000-27b5f442d411115ebaab | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-0a4i-1900000000-b57bcbf0c07e921767af | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-0006-3900000000-1fce5114c61349fc9065 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-014i-0900000000-25c094a4a18cfe463b6b | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-00di-9200000000-e518e242f12e95bbddf9 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive | splash10-00di-9000000000-932dfd009a8e5c41ec38 | View in MoNA |
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1D NMR | 1H NMR Spectrum | Not Available |
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2D NMR | [1H,13C] 2D NMR Spectrum | Not Available |
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References |
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References: |
- Kleijer WJ, Garritsen VH, Linnebank M, Mooyer P, Huijmans JG, Mustonen A, Simola KO, Arslan-Kirchner M, Battini R, Briones P, Cardo E, Mandel H, Tschiedel E, Wanders RJ, Koch HG: Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families. J Inherit Metab Dis. 2002 Sep;25(5):399-410. [12408190 ]
- 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. [19212411 ]
- Michels VV, Beaudet AL: Arginase deficiency in multiple tissues in argininemia. Clin Genet. 1978 Jan;13(1):61-7. [624188 ]
- Glick NR, Snodgrass PJ, Schafer IA: Neonatal argininosuccinic aciduria with normal brain and kidney but absent liver argininosuccinate lyase activity. Am J Hum Genet. 1976 Jan;28(01):22-30. [174426 ]
- Pelli N, Fensom AH, Slade C, Boa F, Mieli-Vergani G, Vergani D: Argininosuccinate lyase: a new autoantigen in liver disease. Clin Exp Immunol. 1998 Dec;114(3):455-61. [9844057 ]
- Scaglia F, Brunetti-Pierri N, Kleppe S, Marini J, Carter S, Garlick P, Jahoor F, O'Brien W, Lee B: Clinical consequences of urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolism. J Nutr. 2004 Oct;134(10 Suppl):2775S-2782S; discussion 2796S-2797S. [15465784 ]
- Cohen BD: Methyl group deficiency and guanidino production in uremia. Mol Cell Biochem. 2003 Feb;244(1-2):31-6. [12701806 ]
- Au WL, Lim TC, Seow DC, Koh PL, Loh NK, Lim MS, Tan IK, Yee WC: Serial diffusion-weighted magnetic resonance imaging in adult-onset citrullinaemia. J Neurol Sci. 2003 May 15;209(1-2):101-4. [12686410 ]
- Wasant P, Srisomsap C, Liammongkolkul S, Svasti J: Urea cycle disorders in Thai infants: a report of 5 cases. J Med Assoc Thai. 2002 Aug;85 Suppl 2:S720-31. [12403252 ]
- Reid Sutton V, Pan Y, Davis EC, Craigen WJ: A mouse model of argininosuccinic aciduria: biochemical characterization. Mol Genet Metab. 2003 Jan;78(1):11-6. [12559843 ]
- Fleisher LD, Rassin DK, Desnick RJ, Salwen HR, Rogers P, Bean M, Gaull GE: Argininosuccinic aciduria: prenatal studies in a family at risk. Am J Hum Genet. 1979 Jul;31(4):439-45. [484552 ]
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Synthesis Reference: |
Hagino, Koji; Nakanishi, Toshihide. Fermentative production of L-arginosuccinic acid. Jpn. Kokai Tokkyo Koho (1980), 3 pp. |
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Material Safety Data Sheet (MSDS) |
Download (PDF) |
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Links |
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External Links: |
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