Overview

  • Product name
    Anti-CPS1 antibody [EPR7493-3]
    See all CPS1 primary antibodies
  • Description
    Rabbit monoclonal [EPR7493-3] to CPS1
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WB, IP, IHC-P, Flow Cyt, ICC/IFmore 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 and HeLa cell lysate. IHC-P: Human liver tissue. Flow Cyt: HeLa cells.
  • 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 ab129076 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 1/1000 - 1/10000. Detects a band of approximately 150 kDa (predicted molecular weight: 165 kDa).
IP 1/10 - 1/100.
IHC-P 1/100 - 1/250. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
Flow Cyt 1/20.
ICC/IF 1/50 - 1/100.

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] (ab129076) at 1/1000 dilution

    Lane 1 : Fetal liver cell lysate
    Lane 2 : Mouse liver cell lysate
    Lane 3 : Rat liver cell lysate
    Lane 4 : HeLa cell lysate

    Predicted band size: 165 kDa
    Observed band size: 150 kDa
    why is the actual band size different from the predicted?

  • ab129076, at 1/100 dilution staining CPS1 in formalin fixed paraffin embedded Human liver tissue by immunohistochemistry
  • Flow cytometry analysis of HeLa (Human cervix adenocarcinoma epithelial cell) cells labeling CPS1 (red) with ab129076 at a 1/20 dilution. Cells were fixed with 4% paraformaldehyde and permeabilized with 90% methanol. A goat anti-rabbit IgG (Alexa Fluor® 488) (ab150077) was used as the secondary antibody at a 1/2000 dilution. Black - Rabbit monoclonal IgG (Black) (ab172730). Blue (unlabeled control) - Cell without incubation with primary antibody and secondary antibody (Blue).

References

This product has been referenced in:
  • Snider NT  et al. Ethanol and Acetaminophen Synergistically Induce Hepatic Aggregation and TCH346-Insensitive Nuclear Translocation of GAPDH. PLoS One 11:e0160982 (2016). Mouse . Read more (PubMed: 27513663) »
See 1 Publication for this product

Customer reviews and Q&As

1-4 of 4 Abreviews or Q&A

Answer

Thank you for your reply.

The exact immunogen sequence for ab129076 is proprietary, but I can inform you that it will bind between amino *.

Please let me know if there is anything else I can help you with.

Read More

Answer

Thank you for your reply.

All of the information regarding the location of the epitopes for ab129076 and ab128942 can be found on the webpage and datasheet for these individual products, links to these are below:

https://www.abcam.com/cps1-antibody-epr7494-ab128942.html
https://www.abcam.com/cps1-antibody-epr7493-ab129076.html



In terms of which domain that epiotpe corresponds to, I would recommend searching the uniprot sequence for CSP1 (http://www.uniprot.org/), as it may be able to supply that information, we do not currently have that in our notes.

If there is anything else I can help you with, please let me know.

Read More

Answer

Thank you for contacting Abcam.

The immunogen sequence for a129076 is closer to the N- terminus rather than the C-terminus and so it would not be suitable for you. However, ab128942, would be suitable for you. The epitope is at the C-terminal region and it will react with human samples:

https://www.abcam.com/CPS1-antibody-EPR7494-ab128942.html.



If there is anything else I can help you with, please let me know.

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Question
Answer

The concentration for ab129076, lot GR87317-2, is 0.131 mg/mL.
I hope this is helpful. Please contact me again if you have any further questions.

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