CPS1 antibody

GeneTex

CPS1 antibody

9

WB: 1:1000 - 1:2000. ICC/IF: 1:50 - 1:200. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.

ImmunoFluorescence, Western Blot, ImmunoCytoChemistry

Research Use Only

Polyclonal

Unconjugated

CPS1

carbamoyl-phosphate synthase 1

carbamoyl-phosphate synthase (ammonia); carbamoyl-phosphate synthase [ammonia], mitochondrial; carbamoyl-phosphate synthase 1, mitochondrial; carbamoylphosphate synthetase I; CPSASE1; GATD6; PHN

Rabbit

IgG

Carbamoyl-phosphate synthase [ammonia], mitochondrial

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]

-20°C or -80°C,2°C to 8°C

12352203