|Other Names||Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, CPSase I, CPS1|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. 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 catalyzessynthesis of carbamoyl phosphate from ammonia and bicarbonate. Thisreaction is the first committed step of the urea cycle, which isimportant in the removal of excess urea from cells. The encodedprotein may also represent a core mitochondrial nucleoid protein.Three transcript variants encoding different isoforms have beenfound for this gene. The shortest isoform may not be localized tothe mitochondrion. Mutations in this gene have been associated withcarbamoyl phosphate synthetase deficiency, susceptibility topersistent pulmonary hypertension, and susceptibility tovenoocclusive 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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