KD-Validated Anti Carbamoyl-phosphate synthase 1 Antibody, clone 23GB2365, Rabbit Monoclonal

The 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]

A synthesized peptide corresponding to a sequence within amino acids 100-166 of human CPS1 [NP_001116105.1].

Solution in PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.

Gently mix before use. Optimal concentrations and conditions for each application should be determined by the user.