CPS1抗体;CPS1 Polyclonal Antibod

Description 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. Specification Aliases:PHN; CPSASE1 Swissprot:P31327 Host/Isotype:Rabbit IgG Antibody Type:Primary antibody Storage:Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. Species Reactivity:Human, Mouse, Rat Immunogen:Synthetic peptide of human CPS1 Formulation:pH7.4 PBS, 0.05% NaN3, 40% Glycerol Application IHC:1/25-1/100 ELISA:1/5000-1/10000