Argininosuccinate synthase
| Argininosuccinate synthase | ||
|---|---|---|
|
||
| Argininosuccinate synthetase tetramer, Thermus thermophilus according to PDB 1J1Z | ||
| Properties of human protein | ||
| Mass / length primary structure | 412 amino acids | |
| Secondary to quaternary structure | Homotetramer | |
| Identifier | ||
| Gene name | ASS1 | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 6.3.4.5 , ligase | |
| Response type | addition | |
| Substrate | ATP + citrulline + aspartate | |
| Products | AMP + diphosphate + argininosuccinate | |
| Occurrence | ||
| Parent taxon | Creature | |
| Orthologue | ||
| human | House mouse | |
| Entrez | 445 | 11898 |
| Ensemble | ENSG00000130707 | ENSMUSG00000076441 |
| UniProt | P00966 | P16460 |
| Refseq (mRNA) | NM_000050 | NM_007494 |
| Refseq (protein) | NP_000041 | NP_031520 |
| Gene locus | Chr 9: 130.44 - 130.5 Mb | Chr 2: 31.47 - 31.52 Mb |
| PubMed search | 445 |
11898
|
The argininosuccinate synthase (ASS) (also argininosuccinate synthetase) is the enzyme that the conversion of aspartate and citrulline to argininosuccinate catalyzed . This reaction takes place in all living things during the biosynthesis of the amino acid arginine and is part of the urea cycle in vertebrates . Mutations in ASS1 - gene are the cause of Citrullinemia Type I .
The regulation of liver-specific ASS expression takes over cAMP - signaling pathways instead. In the Caco-2 cell line , IL-1-beta and nitric oxide act as antagonists of ASA regulation.
Catalyzed reaction
+ 
+ ATP ⇔
⇔ 
+ AMP + PP i
Citrulline and aspartate condense to form arginine succinate.
Individual evidence
- ↑ Homologues at OMA
- ↑ UniProt P00966
- ↑ Engel K, Höhne W, Häberle J: Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene . In: Hum. Mutat. . 30, No. 3, March 2009, pp. 300-7. doi : 10.1002 / humu.20847 . PMID 19006241 .
- ↑ Guei TR, Liu MC, Yang CP, Su TS: Identification of a liver-specific cAMP response element in the human argininosuccinate synthetase gene . In: Biochem Biophys Res Commun . 377, No. 1, December 2008, pp. 257-61. doi : 10.1016 / j.bbrc.2008.09.118 . PMID 18840401 .
- Jump up ↑ Brasse-Lagnel C, Lavoinne A, Fairand A, Vavasseur K, Deniel N, Husson A: Biphasic effect of IL-1beta on the activity of argininosuccinate synthetase in Caco-2 cells. Involvement of nitric oxide production . In: Biochemistry . 88, No. 6, June 2006, pp. 607-12. doi : 10.1016 / j.biochi.2005.11.005 . PMID 16380201 .
Web links
Wikibooks: Urea Cycle - Learning and Teaching Materials
