Pluripotent stem cell-derived bile canaliculi-forming hepatocytes to study genetic liver diseases involving hepatocyte polarity.

Journal of Hepatology(2019)

引用 30|浏览20
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摘要
•Functional cell polarity can be achieved in patient pluripotent stem cell-derived hepatocytes.•This will enable the study of autologous mutant proteins in biliary processes.•The Wilson disease-causing ATP7B-H1069Q mutation prevents its copper-induced polarized redistribution.•MEDNIK syndrome-causing AP1S1 mutations do not cause the expected defect in copper-stimulated redistribution of ATP7B.
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关键词
Wilson disease,Hepatocyte polarity,Pluripotent stem cells,Disease model,MEDNIK,ATPB7,Inherited liver disease
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