New England Journal of Medicine · 1998 · 701 citations · 67 references
MedicineBiochemistryMolecular BiologyHepatologyIntracellular TransportMembrane TraffickingBile FormationBile SecretionHepatic PathophysiologyHepatobiliary DiseaseLiver DiseaseMetabolic Liver DiseaseBile Transport DefectsCholestasis PathophysiologyGenetic Cholestatic DisordersHepatocellular SecretionMembrane Transport SystemsMolecular Mechanisms Of PathogenesisSecretory ApparatusTransporter-mediated Cholestasis
The formation of bile is a vital function, and its impairment by drugs or infectious, autoimmune, metabolic, or genetic disorders results in the syndrome commonly known as cholestasis.1 The secretion of bile normally depends on the function of a number of membrane transport systems in hepatocytes and bile-duct epithelial cells (cholangiocytes) and on the structural and functional integrity of the bile-secretory apparatus. This review summarizes the molecular defects in hepatocellular membrane transporters that are associated with various forms of cholestatic liver disease in humans.Molecular Mechanisms of Bile FormationBile formation is an osmotic secretory process that is driven by . . .
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Congenital Jaundice in Rats with a Mutation in a Multidrug Resistance-Associated Protein Gene
Coen C. Paulusma, Piter J. Bosma, Guido J.R. Zaman et al. · Science · 1996 · 832 citations