This product (Catalog No. DPABH-21726) is a rabbit-derived polyclonal antibody raised against human CPS1. It has been affinity purified by Protein A. 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. DPABH-21726 is suitable for use in IF (immunofluorescence) (1: 25-100) application. DPABH-21726 is supplied as a liquid formulation in pH7.4 PBS, 0.05% sodium azide, 40% Glycerol. For long-term storage, keep at −20 °C. For short-term use, store at 2–8 °C. Avoid repeated freeze–thaw cycles to maintain antibody integrity and performance.
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