L-Argininosuccinic acid lithium salt

SIAL/95148 - analytical standard

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

Empirical Formula (Hill Notation): C₁₀H₁₈N₄O₆ · xLi⁺
Molecular Weight: 290.27 (free acid basis)
Linear Formula: C₁₀H₁₈N₄O₆ · xLi⁺
Product Type: Chemical

Catalog Number	PKG Qty.	Price


45-95148-10MG	10 mg	$167.00 1/EA	Add To Favorites
45-95148-50MG	50 mg	$336.00 1/EA	Add To Favorites

Properties
Descriptions
Safety Info.
Basic Data

application(s)	clinical testing
assay	≥90.0% (HPLC)
form	solid
grade	analytical standard
impurities	≤20% water
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
InChI key	MGJRREWOOITZBB-GEMLJDPKSA-N
optical activity	[α]/D 15±2°, c = 1 in H₂O
shelf life	limited shelf life, expiry date on the label
SMILES string	OC(C[C@@H](C(O)=O)NC(NCCC[C@H](N)C(O)=O)=N)=O.[Li+]
storage temp.	2-8°C

Biochem/physiol Actions:

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.

WGK Germany

WGK 3

Purity	≥90.0% (HPLC)
Storage Temp.	2-8°C
UNSPSC	41116107

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