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- W2279782675 endingPage "496" @default.
- W2279782675 startingPage "474" @default.
- W2279782675 abstract "This chapter outlines the cellular and molecular basis for bile production by the liver and highlights several specific disease entities due to defects in hepatobiliary transport, which lead directly to impaired bile formation. While bile formation is initiated at the canalicular membrane of the hepatocyte, bile is subsequently modified by the regulated secretion and absorption of electrolytes and water by cholangiocytes as it traverses the intrahepatic ducts. These two cell types (hepatocytes and cholangiocytes) comprise a functional unit responsible for bile formation. The contributions and regulation of each are described in the chapter. While disorders targeting hepatocytes lead to hepatitis and cholestasis, those targeting cholangiocytes are almost always cholestatic in nature. The chapter presents several of the genetic causes of cholestasis that are associated with alterations in the activity of the transporters and channels. The progressive familial intrahepatic cholestasis (PFIC) disorders describe a group of rare inherited forms of cholestasis." @default.
- W2279782675 created "2016-06-24" @default.
- W2279782675 creator A5014189033 @default.
- W2279782675 date "2015-11-27" @default.
- W2279782675 modified "2023-10-17" @default.
- W2279782675 title "Bile Secretion and Cholestasis" @default.
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