Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, CPSase I, CPS1
Product 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.1) Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Immunogen
This CPS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 262-291 amino acids from the N-terminal region of human CPS1.
Western blot analysis in Hela cell line lysates (35ug/lane).
Application Notes
For WB starting dilution is: 1:1000:
Additional Information
Form
Liquid
Storage Instructions
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.