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CPS1 Antibody (N-term) Blocking Peptide

Synthetic peptide

     
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Product Information
Primary Accession P31327
Clone Names 100528035
Additional Information
Gene ID 1373
Other Names Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, CPSase I, CPS1
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name CPS1
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. Cell membrane {ECO:0000250|UniProtKB:Q8C196}; Peripheral membrane protein; Extracellular side {ECO:0000250|UniProtKB:Q8C196} Note=Localizes to the cell surface of hepatocytes {ECO:0000250|UniProtKB:Q8C196}
Tissue Location Primarily in the liver and small intestine.
Research Areas
Citations (0)
citation

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Background

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.

References

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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$ 277.78
Cat# BP16053a
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