• Product nameAnti-CPS1 antibody
    See all CPS1 primary antibodies
  • Description
    Mouse monoclonal to CPS1
  • Tested applicationsSuitable for: WB, IHC-P, Flow Cytmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Recombinant fragment, corresponding to amino acids 1400-1501 of Human CPS1



Our Abpromise guarantee covers the use of ab54586 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 165 kDa.
IHC-P Use a concentration of 3 µg/ml.
Flow Cyt Use 1µg for 106 cells. ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.


  • FunctionInvolved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
  • Tissue specificityPrimarily in the liver and small intestine.
  • Involvement in diseaseDefects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]. 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.
    Note=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.
  • Sequence similaritiesContains 2 ATP-grasp domains.
    Contains 1 glutamine amidotransferase type-1 domain.
  • DomainThe type-1 glutamine amidotransferase domain is defective.
  • Cellular localizationMitochondrion.
  • Information by UniProt
  • Database links
  • Alternative names
    • Carbamoyl phosphate synthase [ammonia] antibody
    • Carbamoyl phosphate synthase [ammonia] mitochondrial antibody
    • Carbamoyl phosphate synthase antibody
    • Carbamoyl phosphate synthetase 1 antibody
    • Carbamoyl phosphate synthetase 1 mitochondrial antibody
    • Carbamoyl phosphate synthetase I antibody
    • Carbamoyl-phosphate synthase [ammonia] antibody
    • Carbamoyl-phosphate synthetase I antibody
    • Carbamoylphosphate synthase antibody
    • Carbamoylphosphate synthetase 1 antibody
    • Carbamoylphosphate synthetase I antibody
    • CPS 1 antibody
    • Cps1 antibody
    • CPSase 1 antibody
    • CPSase I antibody
    • CPSASE1 antibody
    • CPSM_HUMAN antibody
    • mitochondrial antibody
    • MS738 antibody
    see all

Anti-CPS1 antibody images

  • CPS1 antibody (ab54586) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human small Intestine.

  • Predicted band size : 165 kDa
    CPS1 antibody (ab54586) at 1ug/lane + HeLa cell lysate at 25ug/lane.
  • Overlay histogram showing HeLa cells stained with ab54586 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab54586, 1µg/1x106 cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG1 [ICIGG1] (ab91353, 2µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed.

References for Anti-CPS1 antibody (ab54586)

ab54586 has not yet been referenced specifically in any publications.

Product Wall

Application Western blot
Loading amount 40 µg
Gel Running Conditions Reduced Denaturing (5% gel)
Sample Chicken Cell lysate - other (DT40 cells (B-cells))
Specification DT40 cells (B-cells)
Treatment treated with paclitaxel
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 25°C

Ms. Serena Clark

Verified customer

Submitted Jul 29 2014