Wilson disease protein ATP7B is localized in the late endosomes in a polarized human hepatocyte cell line

  • Authors:
    • Masaru Harada
    • Hiroto Kumemura
    • Shotaro Sakisaka
    • Shoichiro Shishido
    • Eitaro Taniguchi
    • Takumi Kawaguchi
    • Shinichiro Hanada
    • Hironori Koga
    • Ryukichi Kumashiro
    • Takato Ueno
    • Tatsuo Suganuma
    • Koh Furuta
    • Masayoshi Namba
    • Toshihiro Sugiyama
    • Michio Sata
  • View Affiliations

  • Published online on: March 1, 2003     https://doi.org/10.3892/ijmm.11.3.293
  • Pages: 293-298
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Abstract

Wilson disease is a genetic disorder characterized by the accumulation of copper in the body due to a defect of biliary copper excretion. The gene responsible for Wilson disease has been cloned, however, the precise localization of this gene product ATP7B, a copper-transporting ATPase, is still controversial. We examined the localization of ATP7B by expressing a chimeric protein, ATP7B-tagged with green fluorescent protein (GFP) (GFP-ATP7B), in HEK293, Hep3B and a highly polarized human hepatocyte line (OUMS29). Intracellular organelles were visualized by immunofluorescence microscopy. The effects of bathocuproine disulfonate, a copper chelator, and copper sulfate were examined. GFP-ATP7B colocalized with a late endosome marker, but not with endoplasmic reticulum, Golgi, or lysosome markers in a copper-depleting condition. Treatment with copper sulfate did not affect the localization of ATP7B. ATP7B is localized in the late endosomes in both copper-depleting and copper-loaded conditions. ATP7B seems to translocate copper from the cytosol into the late endosomes, and copper may be excreted to bile via lysosomes. We believe that the disturbed incorporation of copper into the late endosomes caused by mutated ATP7B is the main defect in Wilson disease.

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March 2003
Volume 11 Issue 3

Print ISSN: 1107-3756
Online ISSN:1791-244X

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Spandidos Publications style
Harada M, Kumemura H, Sakisaka S, Shishido S, Taniguchi E, Kawaguchi T, Hanada S, Koga H, Kumashiro R, Ueno T, Ueno T, et al: Wilson disease protein ATP7B is localized in the late endosomes in a polarized human hepatocyte cell line. Int J Mol Med 11: 293-298, 2003.
APA
Harada, M., Kumemura, H., Sakisaka, S., Shishido, S., Taniguchi, E., Kawaguchi, T. ... Sata, M. (2003). Wilson disease protein ATP7B is localized in the late endosomes in a polarized human hepatocyte cell line. International Journal of Molecular Medicine, 11, 293-298. https://doi.org/10.3892/ijmm.11.3.293
MLA
Harada, M., Kumemura, H., Sakisaka, S., Shishido, S., Taniguchi, E., Kawaguchi, T., Hanada, S., Koga, H., Kumashiro, R., Ueno, T., Suganuma, T., Furuta, K., Namba, M., Sugiyama, T., Sata, M."Wilson disease protein ATP7B is localized in the late endosomes in a polarized human hepatocyte cell line". International Journal of Molecular Medicine 11.3 (2003): 293-298.
Chicago
Harada, M., Kumemura, H., Sakisaka, S., Shishido, S., Taniguchi, E., Kawaguchi, T., Hanada, S., Koga, H., Kumashiro, R., Ueno, T., Suganuma, T., Furuta, K., Namba, M., Sugiyama, T., Sata, M."Wilson disease protein ATP7B is localized in the late endosomes in a polarized human hepatocyte cell line". International Journal of Molecular Medicine 11, no. 3 (2003): 293-298. https://doi.org/10.3892/ijmm.11.3.293