Monoclonal Antibody to CPS1 / Carbamoyl-Phosphate Synthetase (Hepatocellular Marker)(Clone : SPM615)

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.

Immunofluorescence (1-2ug/ml); Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT)(Staining of formalin-fixed tissues requires heating tissue sections in 1mM EDTA, pH 7.5-8.5, for 45 min at 95°C followed by cooling at RT for 20 minutes)

For Research Use Only. Not for use in diagnostic/therapeutics procedures.

Subcellular location:	Mitochondrion, Nucleus
Post transnational modification:	Glutarylated. Glutarylation levels increase during fasting. Deglutarylated by SIRT5 at Lys-55, Lys-219, Lys-412, Lys-889, Lys-892, Lys-915, Lys-1360 and Lys-1486, leading to activation.
Tissue Specificity:	Primarily in the liver and small intestine.
BioGrid:	107764. 66 interactions.

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