Description

  • Product name

    Recombinant Human ATP7b protein
  • Expression system

    Wheat germ
  • Accession

  • Protein length

    Protein fragment
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      QLKCYKKPDLERYEAQAHGHMKPLTASQVSVHIGMDDRWRDSPRATPWDQ VSYVSQVSLSSLTSDKPSRHSAAADDDGDKWSLLLNGRDEEQYI
    • Predicted molecular weight

      36 kDa including tags
    • Amino acids

      1372 to 1465

Specifications

Our Abpromise guarantee covers the use of ab152216 in the following tested applications.

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

  • Applications

    SDS-PAGE

    ELISA

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

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

Images

  • 12.5% SDS-PAGE analysis of ab152216 stained with Coomassie Blue.

References

ab152216 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab152216.
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