| Tissue Specificity | Primarily in the liver and small intestine. |
| Post Translational Modifications | Undergoes proteolytic cleavage in the C-terminal region corresponding to the loss of approximately 12 AA residues from the C-terminus. Succinylated at Lys-287 and Lys-1291. Desuccinylated at Lys-1291 by SIRT5, leading to activation. Glutarylated. Glutarylation levels increase during fasting. Deglutarylated by SIRT5 at Lys-55, Lys-219, Lys-412, Lys-889, Lys-892, Lys-915, Lys-1360 and Lys-1486, leading to activation. |
| Function | Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. |
| Protein Name | Carbamoyl-Phosphate Synthase Ammonia - MitochondrialCarbamoyl-Phosphate Synthetase ICpsase I |
| Database Links | Reactome: R-HSA-70635 |
| Cellular Localisation | MitochondrionNucleusNucleolusCell MembranePeripheral Membrane ProteinExtracellular SideLocalizes To The Cell Surface Of Hepatocytes |
| Alternative Antibody Names | Anti-Carbamoyl-Phosphate Synthase Ammonia - Mitochondrial antibodyAnti-Carbamoyl-Phosphate Synthetase I antibodyAnti-Cpsase I antibodyAnti-CPS1 antibody |
Information sourced from Uniprot.org
