Carbamoyl-phosphate synthetase 1 (CPS1); Carbamoylphosphate synthetase 1; CPSase 1; CPSASE1

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

Monoclonal

IgG1

SPM615

Mouse

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. HCCs 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.

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.

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.

Small volumes of anti-CPS1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Immunofluorescence (IF), Immunohistochemistry (IHC) Formalin

Immunofluorescence (0.5-1.0ug/ml)
Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT)
(Staining of formalin-fixed tissues requires boiling tissue sections in 1mM EDTA, pH 7.5-8.5, for 10-20 min followed by cooling at RT for 20 minutes)

Optimal dilution for a specific application should be determined.

Formalin-fixed, paraffin-embedded human Hepatocellular Carcinoma stained with CPS1 Monoclonal Antibody (SPM615).

~165kDa

carbamoyl-phosphate synthase 1

carbamoyl-phosphate synthase [ammonia], mitochondrial

Carbamoyl-phosphate synthase [ammonia], mitochondrial

CPSase I??[Similar Products]

CPSM_HUMAN

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]

CPS1: Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D). CPS1D is an autosomal recessive disorder of the urea cycle causing hyperammonemia. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation. Genetic variations in CPS1 influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Nucleolus; EC 6.3.4.16; Ligase; Amino Acid Metabolism - alanine, aspartate and glutamate; Mitochondrial; Amino Acid Metabolism - arginine and proline; Energy Metabolism - nitrogen

Chromosomal Location of Human Ortholog: 2q35

Cellular Component: mitochondrial matrix

Molecular Function: aspartate carbamoyltransferase activity; carbamoyl-phosphate synthase (ammonia) activity; carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity; dihydroorotase activity; protein binding

Biological Process: 'de novo' pyrimidine base biosynthetic process; arginine biosynthetic process; glycogen catabolic process; homocysteine metabolic process; nitric oxide metabolic process; positive regulation of vasodilation; response to lipopolysaccharide; triacylglycerol catabolic process; urea cycle

Disease: Carbamoyl Phosphate Synthetase I Deficiency, Hyperammonemia Due To; Pulmonary Hypertension, Neonatal, Susceptibility To

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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