Click here to close
Hello! We notice that you are using Internet Explorer, which is not supported by Xenbase and may cause the site to display incorrectly.
We suggest using a current version of Chrome,
FireFox, or Safari.
Delivery of the Cu-transporting ATPase ATP7B to the plasma membrane in Xenopus oocytes.
Lörinczi E
,
Tsivkovskii R
,
Haase W
,
Bamberg E
,
Lutsenko S
,
Friedrich T
.
???displayArticle.abstract???
Cu-transporting ATPase ATP7B (Wilson disease protein) is essential for the maintenance of intracellular copper concentration. In hepatocytes, ATP7B is required for copper excretion, which is thought to occur via a transient delivery of the ATP7B- and copper-containing vesicles to the apical membrane. The currently available experimental systems do not allow analysis of ATP7B at the cell surface. Using epitope insertion, we identified an extracellular loop into which the HA-epitope can be introduced without inhibiting ATP7B activity. The HA-tagged ATP7B was expressed in Xenopus oocytes and the presence of ATP7B at the plasma membrane was demonstrated by electron microscopy, freeze-fracture experiments, and surface luminescence measurements in intact cells. Neither the deletion of the entire N-terminal copper-binding domain nor the inactivating mutation of catalytic Asp1027 affected delivery to the plasma membrane of oocytes. In contrast, surface targeting was decreased for the ATP7B variants with mutations in the ATP-binding site or the intra-membrane copper-binding site, suggesting that ligand-stabilized conformation(s) are important for ATP7B trafficking. The developed system provides significant advantages for studies that require access to both sides of ATP7B in the membrane.
Axelsen,
Evolution of substrate specificities in the P-type ATPase superfamily.
1998, Pubmed
Axelsen,
Evolution of substrate specificities in the P-type ATPase superfamily.
1998,
Pubmed
Beggah,
beta-subunit assembly is essential for the correct packing and the stable membrane insertion of the H,K-ATPase alpha-subunit.
1999,
Pubmed
,
Xenbase
Beguin,
Phosphorylation of the Na,K-ATPase alpha-subunit by protein kinase A and C in vitro and in intact cells. Identification of a novel motif for PKC-mediated phosphorylation.
1994,
Pubmed
,
Xenbase
Beguin,
Structural domains implicated in ER degradation of alpha subunits of Na,K-ATPase.
1997,
Pubmed
,
Xenbase
Bissig,
Expression of the human Menkes ATPase in Xenopus laevis oocytes.
2001,
Pubmed
,
Xenbase
Bull,
The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene.
1993,
Pubmed
Cater,
Intracellular trafficking of the human Wilson protein: the role of the six N-terminal metal-binding sites.
2004,
Pubmed
Compston,
Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, by S. A. Kinnier Wilson, (From the National Hospital, and the Laboratory of the National Hospital, Queen Square, London) Brain 1912: 34; 295-509.
2009,
Pubmed
Czaja,
Molecular studies of ceruloplasmin deficiency in Wilson's disease.
1987,
Pubmed
Dmitriev,
Solution structure of the N-domain of Wilson disease protein: distinct nucleotide-binding environment and effects of disease mutations.
2006,
Pubmed
Drumm,
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
1991,
Pubmed
,
Xenbase
Forbes,
Copper-dependent trafficking of Wilson disease mutant ATP7B proteins.
2000,
Pubmed
Fujimoto,
A simple and reliable quick-freezing/freeze-fracturing procedure.
1997,
Pubmed
Gatto,
Heterologous expression of Na(+)-K(+)-ATPase in insect cells: intracellular distribution of pump subunits.
2001,
Pubmed
Guo,
NH2-terminal signals in ATP7B Cu-ATPase mediate its Cu-dependent anterograde traffic in polarized hepatic cells.
2005,
Pubmed
Hu,
Expression of an active Na,K-ATPase with an alpha-subunit lacking all twenty-three native cysteine residues.
2000,
Pubmed
Laemmli,
Cleavage of structural proteins during the assembly of the head of bacteriophage T4.
1970,
Pubmed
Lorenz,
Heteromultimeric CLC chloride channels with novel properties.
1996,
Pubmed
,
Xenbase
Luckow,
Efficient generation of infectious recombinant baculoviruses by site-specific transposon-mediated insertion of foreign genes into a baculovirus genome propagated in Escherichia coli.
1993,
Pubmed
Lutsenko,
Localization of the Wilson's disease protein product to mitochondria.
1998,
Pubmed
Melchers,
Cloning and membrane topology of a P type ATPase from Helicobacter pylori.
1996,
Pubmed
Melchers,
Membrane topology of CadA homologous P-type ATPase of Helicobacter pylori as determined by expression of phoA fusions in Escherichia coli and the positive inside rule.
1999,
Pubmed
Mercer,
Copper-induced trafficking of the cU-ATPases: a key mechanism for copper homeostasis.
2003,
Pubmed
Nomizu,
Zinc, iron, and copper contents of Xenopus laevis oocytes and embryos.
1993,
Pubmed
,
Xenbase
Payne,
Functional expression of the Wilson disease protein reveals mislocalization and impaired copper-dependent trafficking of the common H1069Q mutation.
1998,
Pubmed
Petris,
Copper-regulated trafficking of the Menkes disease copper ATPase is associated with formation of a phosphorylated catalytic intermediate.
2002,
Pubmed
Roelofsen,
Copper-induced apical trafficking of ATP7B in polarized hepatoma cells provides a mechanism for biliary copper excretion.
2000,
Pubmed
Sarkadi,
Molecular characterization of the in situ red cell membrane calcium pump by limited proteolysis.
1986,
Pubmed
Schaefer,
Hepatocyte-specific localization and copper-dependent trafficking of the Wilson's disease protein in the liver.
1999,
Pubmed
Sokac,
Cdc42-dependent actin polymerization during compensatory endocytosis in Xenopus eggs.
2003,
Pubmed
,
Xenbase
Tanzi,
The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene.
1993,
Pubmed
Tsivkovskii,
Functional properties of the copper-transporting ATPase ATP7B (the Wilson's disease protein) expressed in insect cells.
2002,
Pubmed
Tsivkovskii,
The role of the invariant His-1069 in folding and function of the Wilson's disease protein, the human copper-transporting ATPase ATP7B.
2003,
Pubmed
Voskoboinik,
Mutational analysis of the Menkes copper P-type ATPase (ATP7A).
2003,
Pubmed
Yoo,
Cell surface expression of the ROMK (Kir 1.1) channel is regulated by the aldosterone-induced kinase, SGK-1, and protein kinase A.
2003,
Pubmed
,
Xenbase
Zeng,
Evidence for endocytosis of ROMK potassium channel via clathrin-coated vesicles.
2002,
Pubmed
,
Xenbase
Zerangue,
A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels.
1999,
Pubmed
,
Xenbase