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.
Applications:Suitable for use in Western Blot, ELISA
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with horseradish peroxidase (HRP).
Specificity:Human
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu
Immunogen:CPS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 261-291 amino acids from the N-terminal region of human CPS1.