034177
CPS1, NT (CPS1, Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I)
Size
£696.00
- SKU:
- 034177
- Purity:
- Purified by Protein A affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- 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.
- Format:
- Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
- Extra Details:
- 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 Recommended Dilution: ELISA: 1:1,000 Western Blot: 1:100-500 Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice