Overview

  • Product name

    Anti-ATP7b antibody - C-terminal
    See all ATP7b primary antibodies
  • Description

    Rabbit polyclonal to ATP7b - C-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Rat
    Predicted to work with: Mouse, Human
  • Immunogen

    Synthetic peptide within Human ATP7b aa 1410-1460 (C terminal) conjugated to keyhole limpet haemocyanin. The exact sequence is proprietary.
    Database link: P35670

  • Positive control

    • Rat brain tissue

Properties

Applications

Our Abpromise guarantee covers the use of ab217299 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 1/100 - 1/500.

Target

  • Function

    Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
  • Tissue specificity

    Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.
  • Involvement in disease

    Defects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.
  • Sequence similarities

    Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
    Contains 6 HMA domains.
  • Post-translational
    modifications

    Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.
  • Cellular localization

    Cytoplasm; Mitochondrion and Golgi apparatus > trans-Golgi network membrane. Predominantly found in the trans-Golgi network (TGN). Not redistributed to the plasma membrane in response to elevated copper levels.
  • Information by UniProt
  • Database links

  • Alternative names

    • ATP7B antibody
    • ATP7B_HUMAN antibody
    • ATPase, Cu(2+) transporting, beta polypeptide antibody
    • ATPase, Cu++ transporting, beta polypeptide antibody
    • Copper pump 2 antibody
    • Copper transporting ATPase 2 antibody
    • PWD antibody
    • Toxic milk antibody
    • tx antibody
    • WC1 antibody
    • WD antibody
    • Wilson disease associated protein antibody
    • Wilson disease-associated protein antibody
    • WND antibody
    • WND/140 kDa antibody
    see all

Images

  • Immunohistochemical analysis of formalin-fixed and paraffin-embedded rat brain tissue labeling ATP7b with ab217299 at 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.

References

ab217299 has not yet been referenced specifically in any publications.

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