argininosuccinate

argininosuccinate

/ar·gi·ni·no·suc·cin·ate/ (ahr″jĭ-ne″no-suk´sĭ-nāt) the anionic form of argininosuccinic acid.
References in periodicals archive ?
Tumor necrosis factor-reduces argininosuccinate symthase expression and nitric oxide production in aorticendothelial cells.
M2 EQUITYBITES-September 21, 2017-PhaseRx wins US FDA's orphan drug designation for PRX-ASL for argininosuccinate lyase deficiency
M2 PHARMA-September 21, 2017-FDA Grants Orphan Drug Designation to PhaseRx for PRX-ASL for Treatment of Argininosuccinate Lyase Deficiency
These enzymes are carbamoyl phosphate synthetase 1 (CPS1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase and N-acetylglutamate synthetase.
Concentrations of 6 other amino acids including alloisoleucine, homocystine, cystathionine, cystine, argininosuccinate, [alpha]-aminoadipic acid, were not detectable (< 1 [micro]mol/L) in blood spot extracts.
The ASS1 gene provides instructions for making argininosuccinate synthase 1, which is responsible for the third step of the urea cycle.
We have studied the combination of L-CAV with VIN and PTX in three cancer cell lines representing cells with high argininosuccinate synthase (ASS) expression (HeLa) and cells with low ASS expression (Hep G2 and SK-HEP-1 cells).
Cit is transported out from mitochondrion and bound to aspartate by argininosuccinate synthase, and aspartate is provided by the mitochondrial transporter citrin.
Spot 12 contained a mixture of four proteins: argininosuccinate synthase (nine peptides), elongation factor 1-alpha (two peptides), alanine aminotransferase2 (two peptides), and 26S protease regulatory subunit6B homolog (five peptides, also found in other spots) (Table 1, peptide data not shown).
Additional galU genes were found adjacent to the gene argH encoding argininosuccinate lyase in the sequenced genomes from isolates a275, 29031, H-ASH/87, and H-ZANE/83, and although the genes were conserved, they were not identical to the galU genes found within the cps locus.
After demonstrating hyperammonaemia, a specific diagnosis can be made by analysing plasma amino acids, including citrulline and argininosuccinate, and by measuring orotate in urine.