Mouse Monoclonal antibody [2E4AE11] to CPS1The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
Immunogen
Human liver mitochondria.
Key Feature
Clonality
Monoclonal
Isotype
IgG1
Clone Number
2E4AE11
Host Species
Mouse
Tested Applications
ICC/IFIP
ICC/IF:1:200~1:500 IP:1:100~1:500 Notes:Optimal dilutions/concentrations should be determined by the researcher.
Species Reactivity
HumanMouseRatBovine
Concentration
1mg/ml (Please refer to the vial label for the specific concentration)