IHC-P:0.25-0.5ug/ml for 30 minutes at RT.
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.
Prepared in 10mM PBS with 0.05% BSA and 0.05% azide.
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
Recombinant human CPS1 protein
Ab purified from Bioreactor Concentrate by Protein A/G
For laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
Carbamoyl-Phosphate Synthase 1,Cpsase1,Phn,Cps1
The mitochondrial enzyme encoded by this gene 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. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
GTX34613 IHC-P Image
Formalin-fixed, paraffin-embedded human Hepatocellular Carcinoma stained with CPS1 Monoclonal Antibody (CPS1/1022).