Premium
Biliary excretion of copper in LEC rat after introduction of copper transporting P‐type ATPase, ATP7B
Author(s) -
Terada Kunihiko,
Aiba Namiko,
Yang Xiao-Li,
Iida Masatake,
Nakai Michio,
Miura Naoyuki,
Sugiyama Toshihiro
Publication year - 1999
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/s0014-5793(99)00319-1
Subject(s) - copper , atpase , chemistry , excretion , copper metabolism , medicine , biochemistry , enzyme , organic chemistry
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.