Overview

  • Product name

    Anti-CPS1 antibody [EPR7493-3] (HRP)
    See all CPS1 primary antibodies
  • Description

    Rabbit monoclonal [EPR7493-3] to CPS1 (HRP)
  • Host species

    Rabbit
  • Conjugation

    HRP
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human CPS1 aa 100-200. The exact sequence is proprietary.

  • Positive control

    • WB: Human fetal liver, mouse liver and rat liver tissue lysates. IHC-P: normal human liver tissue sections.
  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab198969 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 1/5000. Detects a band of approximately 165 kDa (predicted molecular weight: 165 kDa).
IHC-P 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • Function

    Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
  • Tissue specificity

    Primarily in the liver and small intestine.
  • Involvement in disease

    Defects 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 similarities

    Contains 2 ATP-grasp domains.
    Contains 1 glutamine amidotransferase type-1 domain.
  • Domain

    The type-1 glutamine amidotransferase domain is defective.
  • Cellular localization

    Mitochondrion.
  • 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

Images

  • All lanes : Anti-CPS1 antibody [EPR7493-3] (HRP) (ab198969) at 1/5000 dilution

    Lane 1 : Fetal Liver (Human) Normal Tissue Lysate
    Lane 2 : Liver (Mouse) Tissue Lysate
    Lane 3 : Liver (Rat) Tissue Lysate

    Lysates/proteins at 10 µg per lane.

    Developed using the ECL technique.

    Performed under reducing conditions.

    Predicted band size: 165 kDa
    Observed band size: 165 kDa


    Exposure time: 30 seconds


    This blot was produced using a 4-12% Bis-tris gel under the MOPS buffer system. The gel was run at 200V for 50 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 2% Bovine Serum Albumin before being incubated with ab198969 overnight at 4°C. Antibody binding was visualised using ECL development solution ab133406.

  • IHC image of CPS1 staining in a section of formalin-fixed paraffin-embedded normal human liver tissue*, performed on a Leica BOND. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20mins. The section was then incubated with ab198969 at 1/100 dilution, for 15 mins at room temperature. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX. The inset negative control image is taken from an identical assay without primary antibody.

    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.

    *Tissue obtained from the Human Research Tissue Bank, supported by the NIHR Cambridge Biomedical Research Centre.

References

ab198969 has not yet been referenced specifically in any publications.

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