This product was discontinued; sorry for the inconvenience. We recommend the following: Cat.# A69451.
BackgroundThe 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.
DescriptionCPS1 Polyclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
SpecificityHuman, Mouse, Rat
IsotypeIgG
Uniprot IDP31327
PurificationAffinity Purified
ImmunogenRecombinant protein of human CPS1.
StorageShipped at 4°C. Store at -20°C. Avoid freeze / thaw cycles.
Alternative NamesCPS1; CPSASE1; PHN
ApplicationWB, IF; Recommended dilution: WB 1:1000 - 1:2000, IF 1:50 - 1:200