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Vol. 273, Issue 3, 1815-1820, January 16, 1998
From the Wilson's disease, an autosomal recessive
disorder, is characterized by the excessive accumulation of copper in
the liver. WND (ATP7B) gene, which encodes a
putative copper transporting P-type ATPase, is defective in the
patients. To investigate the in vivo function of WND
protein as well as its intracellular localization, WND
cDNA was introduced to the Long-Evans Cinnamon rat, known as
a rodent model for Wilson's disease, by recombinant
adenovirus-mediated gene delivery. An immunofluorescent study and a
subcellular fractionation study revealed the transgene expression in
liver and its localization to the Golgi apparatus. Moreover, since the
synthesis of holoceruloplasmin is disturbed in the Long-Evans Cinnamon
rat, the plasma level of holoceruloplasmin, oxidase-active and
copper-bound form, was examined to evaluate the function of WND protein
with respect to the copper transport. Consequently, the appearance of
holoceruloplasmin in plasma was confirmed by Western blot analysis and
plasma measurements for the oxidase activity and the copper content.
These findings indicate that introduced WND protein may function in the
copper transport coupled with the synthesis of ceruloplasmin and that the Golgi apparatus is the likely site for WND protein to manifest its
function.
Restoration of Holoceruloplasmin Synthesis in LEC Rat after
Infusion of Recombinant Adenovirus Bearing WND cDNA
,,,,, and
Department of Biochemistry and the
¶ Department of Surgery, Akita University School of Medicine,
Hondo 1-1-1, Akita 010, Japan and § Discovery Research
Laboratories III, Sumitomo Pharmaceuticals Research Center,
3-1-98 Kasugadenaka, Konohanaku, Osaka 554, Japan
Copyright © 1998 by The American Society for Biochemistry and Molecular Biology, Inc.
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