|Application ||WB, E|
|Other Accession||NP_001116105.1, NP_001866.2, NP_001116106.1|
|Calculated MW||164939 Da|
|Antigen Region||262-291 aa|
|Other Names||Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, CPSase I, CPS1|
|Target/Specificity||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.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||CPS1 Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.|
|Cellular Location||Mitochondrion. Nucleus, nucleolus|
|Tissue Location||Primarily in the liver and small intestine.|
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Provided below are standard protocols that you may find useful for product applications.
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.
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Jia, P., et al. Schizophr. Res. 122 (1-3), 38-42 (2010) :
Pekkala, S., et al. Hum. Mutat. 31(7):801-808(2010)
Huo, R., et al. J. Biochem. Mol. Biol. 38(1):28-33(2005)
Hoshide, R., et al. Genomics 28(1):124-125(1995)
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