Biliary excretion of copper in LEC rat after introduction of copper transporting P‐type ATPase, ATP7B

FEBS Letters

Miura, Naoyuki²  Terada, Kunihiko²  Yang, Xiao-Li²  Nakai, Michio¹  Aiba, Namiko²  Sugiyama, Toshihiro²  Iida, Masatake² 

[1] Discovery Research Laboratories III, Sumitomo Pharmaceuticals Research Center, 3-1-98 Kasugadenaka, Konohanaku, Osaka 554-0022, Japan;Department of Biochemistry, Akita University School of Medicine, Hondo 1-1-1, Akita 010-8543, Japan

关键词: Copper transport;    ATP7B;    Bile;    Wilson's disease;    Long Evans Cinnamon rat;    LEC;    Long-Evans Cinnamon;    CPN;    ceruloplasmin;    pfu;    plaque forming unit;    PAGE;    polyacrylamide gel electrophoresis;   

DOI  :  10.1016/S0014-5793(99)00319-1

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Wilson's disease, an autosomal recessive disorder, is characterized by the excessive accumulation of hepatic copper that results from reduced biliary copper excretion and disturbed incorporation of copper into ceruloplasmin. The ATP7B gene, responsible for the disease, encodes a copper transporting P-type ATPase. We previously demonstrated the involvement of ATP7B in hepatic copper secretion into plasma after the introduction of ATP7B into the Long-Evans Cinnamon (LEC) rat, a rodent model of Wilson's disease. In this study we found the increased copper contents of the hepatic lysosomal fractions and bile in the LEC rats after ATP7B introduction, indicating the participation of ATP7B in the biliary excretory pathway for copper.

【 授权许可】

Unknown