• Product nameAnti-CPS1 antibody [2E4AE11]
    See all CPS1 primary antibodies
  • Description
    Mouse monoclonal [2E4AE11] to CPS1
  • Tested applicationsSuitable for: IHC-P, Flow Cyt, ICC, IPmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Tissue, cells or virus corresponding to CPS1.

  • Positive control
    • HDFn and HeLa cells; Human liver mitochondria lysate.
  • General notes

    Immunogen information: Human liver mitochodnria was used to generate this antibody. The exact immunogen sequence is unknown.

    Product was previously marketed under the MitoSciences sub-brand.



Our Abpromise guarantee covers the use of ab110303 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
IHC-P Use a concentration of 10 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
Flow Cyt Use a concentration of 1 µg/ml. ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
ICC Use a concentration of 10 µg/ml.
IP Use at an assay dependent concentration.


  • 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 [2E4AE11] images

  • Immunocytochemistry analysis using ab110303 at 10µg/ml staining CPS1 in HDFn cells (paraformaldehyde fixed and Triton X-100 permeabilized). The secondary antibody was (red) Alexa Fluor® 594 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1h. 10% Goat serum was used as the blocking agent for all blocking steps. DAPI was used to stain the cell nuclei (blue). Target protein locates mainly in mitochondria.
  • Detection of ab110303 by immunoprecipitation staining of 164kDa CPS1, a liver mitochondrial marker in human liver mitochondrial lysate.
  • Flow cytometric analysis using ab110303 at 1µg/ml staining CPS1 in HeLa cells (blue). Isotype control antibody (red).
  • IHC image of ab110303 staining in human duodenum formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab110303, 10µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

    For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

References for Anti-CPS1 antibody [2E4AE11] (ab110303)

This product has been referenced in:
  • Li J  et al. Detection of circulating tumor cells in hepatocellular carcinoma using antibodies against asialoglycoprotein receptor, carbamoyl phosphate synthetase 1 and pan-cytokeratin. PLoS One 9:e96185 (2014). Flow Cyt ; Human . Read more (PubMed: 24763545) »

See 1 Publication for this product

Product Wall

Application IHC - Wholemount
Sample Human Tissue (Liver)
Specification Liver

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Submitted Nov 16 2015