EC Number   |
General Information |
Reference |
|---|
    6.3.4.5 | malfunction |
a cell line lacking two of three LdASS-/-/+ alleles, with greatly reduced argininosuccinate synthase activity, is generated by a double-targeted gene replacement strategy in Leishmania donovani promastigote |
-, 749045 |
| 6.3.4.5 | malfunction |
deficiency of the enzyme may result in citrullinemia type I |
703969 |
| 6.3.4.5 | malfunction |
enzyme deficency results in congenital urea cycle disorders |
705506 |
| 6.3.4.5 | malfunction |
epigenetic silencing triggers apoptosis in arginine auxotroph tumours |
704078 |
| 6.3.4.5 | malfunction |
malfunction results in citrullinemia type 1 argininosuccinate argininosuccinate synthetase deficiency |
703251 |
| 6.3.4.5 | malfunction |
mutation results in citrullinemia type I |
703248 |
| 6.3.4.5 | metabolism |
Sulfolobus solfataricus lacks ornithine acetyltransferase and thus forms N-acetylglutamate exclusively via the energetically less favourable reaction catalysed by N-acetylglutamate synthase, investing 1 mol of acetyl CoA per mol of N-acetyl intermediate synthesized |
-, 725629 |
| 6.3.4.5 | metabolism |
the enzyme plays an important role as the rate-limiting step required for converting L-citrulline to argininosuccinate to provide arginine |
-, 749045 |
| 6.3.4.5 | physiological function |
argininosuccinate synthase 1 (ASS1), is directly transactivated by p53 in response to genotoxic stress, resulting in the rearrangement of arginine metabolism. x-Ray irradiation promotes the systemic induction of Ass1 and concomitantly increases plasma arginine levels in p53+/+ mice but not in p53-/- mice. Ass1+/- mice exhibit hypersensitivity to whole-body irradiation owing to increased apoptosis in the small intestinal crypts. ASS1 plays a pivotal role in limiting Akt phosphorylation. Aberrant activation of Akt resulting from ASS1 loss disrupts Akt-mediated cell survival signaling activity under genotoxic stress |
746432 |
| 6.3.4.5 | physiological function |
argininosuccinate synthase ASS1 is significantly downregulated in the urine of 30 depressed subjects while remaining unchanged in the plasma |
744740 |
