Terada K, Aiba N, Yang X L, Iida M, Nakai M, Miura N, Sugiyama T
Department of Biochemistry, Akita University School of Medicine, Japan.
FEBS Lett. 1999 Apr 1;448(1):53-6. doi: 10.1016/s0014-5793(99)00319-1.
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.
威尔逊氏病是一种常染色体隐性疾病，其特征是肝脏铜过量蓄积，这是由于胆汁铜排泄减少以及铜掺入铜蓝蛋白过程紊乱所致。导致该病的ATP7B基因编码一种铜转运P型ATP酶。我们之前通过将ATP7B导入威尔逊氏病啮齿动物模型长-伊文斯肉桂色大鼠（LEC大鼠），证明了ATP7B参与肝脏铜分泌入血浆的过程。在本研究中，我们发现导入ATP7B后，LEC大鼠肝脏溶酶体组分和胆汁中的铜含量增加，这表明ATP7B参与了铜的胆汁排泄途径。
