Thermo Fisher Scientific CPS1 Monoclonal Antibody (2E4AE11)

Applications and Tested Dilution

Product Specifications

Target Information

The mitochondrial enzyme encoded by this gene catalyzes the synthesis of carbamoyl phosphate from ammonia and bicarbonate, representing the first committed step of the urea cycle. This process is critical for the removal of excess urea from cells. The encoded protein may also function as a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been identified, with the shortest isoform potentially not localized to the mitochondrion. Mutations in this gene are associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and venoocclusive disease following bone marrow transplantation.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.