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73097

Sigma-Aldrich

L-Argininosuccinic acid lithium salt

≥95% (TLC)

Synonyme(s) :

N-{{[(4S)-4-Amino-4-carboxybutyl]amino}iminomethyl}-L-aspartic acid lithium salt, Lithium L-argininosuccinate

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About This Item

Formule empirique (notation de Hill):
C10H18N4O6 · xLi+
Poids moléculaire :
290.27 (free acid basis)
Numéro Beilstein :
6933810
Code UNSPSC :
12352209
ID de substance PubChem :
329764608
Nomenclature NACRES :
NA.26

Niveau de qualité

100

Pureté

≥70% (qNMR)
≥95% (TLC)

Forme

powder or crystals

Activité optique

[α]/D 15±2°, c = 1 in H2O

Impuretés

≤20% water

Couleur

white to off-white

Température de stockage

2-8°C

Chaîne SMILES 

OC(C[C@@H](C(O)=O)NC(NCCC[C@H](N)C(O)=O)=N)=O.[Li+]

InChI

1S/C10H18N4O6.Li/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);/q;+1/t5-,6-;/m0./s1

Clé InChI

MGJRREWOOITZBB-GEMLJDPKSA-N

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Actions biochimiques/physiologiques

Arginosuccinic acid is a basic amino acid, which is synthesized by some cells from citrulline, aspartic acid and used as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The reaction is catalyzed by the enzyme 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.

Code de la classe de stockage

11 - Combustible Solids

Classe de danger pour l'eau (WGK)

WGK 3

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Wing Lok Au et al.
Journal of the neurological sciences, 209(1-2), 101-104 (2003-04-11)
A 25-year-old Chinese man presented with a 2-year history of recurrent coma. His plasma ammonia level was extremely elevated, with raised citrulline level and absence of argininosuccinic acid. Adult-onset citrullinaemia, a condition rarely reported outside the Japanese population, was diagnosed.
L D Fleisher et al.
American journal of human genetics, 31(4), 439-445 (1979-07-01)
We have monitored two successive pregnancies in a family which we found to be at risk for argininosuccinic aciduria. We measured argininosuccinic acid (ASA) concentrations in amniotic fluid and utilized an indirect assay of ASA lyase activity in cultured amniotic
Pornswan Wasant et al.
Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 85 Suppl 2, S720-S731 (2002-10-31)
Urea Cycle Disorders (UCD) is an inborn error of urea synthesis in which ammonium and other nitrogenous precursors of urea accumulate leading to episodic coma and a high mortality rate. Therapy with peritoneal dialysis, essential amino acids or their nitrogen-free
W J Kleijer et al.
Journal of inherited metabolic disease, 25(5), 399-410 (2002-11-01)
A biochemical variant of argininosuccinate lyase deficiency, found in five individuals, is introduced. In comparison to classical patients, the variant cases of argininosuccinate lyase deficiency were characterized by residual enzyme activity as measured by the incorporation of [14C]citrulline into proteins.
Rachel S Carling et al.
Clinical chemistry and laboratory medicine, 58(5), 758-768 (2019-10-18)
Background Quantification of plasma amino acids is key to the diagnosis of inherited defects of amino acid synthesis, catabolism and transport, many of which present as clinical emergencies. The utility of this test is limited by the long analysis time
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