CPS1 / Carbamoyl-Phosphate Synthetase (Hepatocellular Marker) Antibody - With BSA and Azide
Mouse Monoclonal Antibody [Clone CPS1/1022 ]
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| IHC, IF |
---|---|
Primary Accession | P31327 |
Other Accession | 1373, 149252 |
Reactivity | Human, Dog |
Host | Mouse |
Clonality | Monoclonal |
Isotype | Mouse / IgG1 |
Clone Names | CPS1/1022 |
Calculated MW | ~165kDa |
Gene ID | 1373 |
---|---|
Other Names | Carbamoyl-phosphate synthase [ammonia], mitochondrial, 6.3.4.16, Carbamoyl-phosphate synthetase I, CPSase I, CPS1 |
Storage | Store at 2 to 8°C.Antibody is stable for 24 months. |
Precautions | CPS1 / Carbamoyl-Phosphate Synthetase (Hepatocellular Marker) Antibody - With BSA and Azide is for research use only and not for use in diagnostic or therapeutic procedures. |
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. |
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Background
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.ĀDeficiency 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.
References
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. | Fan, Z., et al. Mod. Pathol 2003; 16: 137-144, 2003. |
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