CPS1 / Carbamoyl-Phosphate Synthetase (Hepatocellular Marker) [CPS1/1022]

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Summary

This MAb recognizes a protein of 165kDa, identified as carbamoyl phosphate synthetase 1 (CPS1). This mitochondrial enzyme 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.Deficiency of CPS1 is an autosomal recessive disorder that causes hyperammonemia. CPS1 is a hepatocyte specific protein that localizes to the mitochondria of hepatocytes. It is a sensitive marker for distinguishing hepatocellular carcinomas (HCC) from other metastatic carcinomas as well as cholangio-carcinomas. HCC's occur primarily in the stomach, but they are also found in many other organs. CPS1 may also be a useful marker for intestinal metaplasia. Reportedly, strong expression of CPS1 correlates with smaller tumor size and longer patient survival. Occasionally, CPS1 is also found in gastric carcinomas as well as in a few other non-hepatic tumors.

Product Properties & Targets

Host
Applications
Species Reactivity
Research Areas
Isotype
Cellular Localization
Mitochondrion, Nucleolus, Nucleus
Light Chain
Gene Name
Positive Control
HeLa cells. Liver or Hepatocellular Carcinoma (HCC).
Immunogen
Recombinant human CPS1 protein
Alternate Names
Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, Carbamoyl-phosphate synthetase 1 (CPS1); Carbamoylphosphate synthetase 1; CPSase 1; CPSASE1

Database Links

Entrez Gene ID
Unigene
SwissProt

Additional Information

Clone
CPS1/1022
Human Gene Symbol
CPS1
Chromosome Location
2q35
Mol. Weight of Antigen
~165kDa

Functions

  • Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.

Known Applications & Suggested Dilutions

  • Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT)(Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95&degC followed by cooling at RT for 20 minutes)
  • Optimal dilution for a specific application should be determined.

Key References

  • Fan, Z., et al. Mod. Pathol 2003; 16: 137-144, 2003.
  • Haraguchi, Y., et al. 1991. Cloning and sequence of a cDNA encoding human carbamyl phosphate synthetase I: molecular analysis of hyperam- monemia. Gene 107: 335-340.
  • Ramos-Vara, J.A., et al. Histochem 2002; J. 34: 397-401.

Storage & Stability

Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Limitations

This antibody is available for research use only and is not approved for use in diagnosis.

Supplied as

200ug/ml of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Warranty

There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.

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