4348 T3D4294 Argininosuccinic acid 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. 2387-71-5 16950 C10H18N4O6 White powder. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. 2014-08-29T06:16:32Z 2026-04-02T23:52:12Z Argininosuccinic acid C03406 15682 DB02267 true N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O C10H18N4O6 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)/t5-,6-/m0/s1 KDZOASGQNOPSCU-WDSKDSINSA-N 290.2731 290.122634328 Endogenous Solid HMDB00052 16059 <p>Steven S. Gross, Owen W. Griffith, &#8220;Preventing conversion of citrulline to argininosuccinate to limit pathological nitric oxide overproduction.&#8221; U.S. Patent US5545625, issued January, 1995.</p> CHEM003254 DTXSID80178574 NS00014794 185.82999999999998 75.3116 27.598868203245992 9 10 7 2.1362975685976218 12.386778734858158 -1 0 -3.25 -2.80 4.56e-01 g/l