Concepedia

Molecular Pathogenesis of Cholestasis

Michael Trauner, Peter J. Meier, James L. Boyer

New England Journal of Medicine · 1998 · 698 citations · 67 references

Concepts

Abstract

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 . . .

References

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Mutations in the <i>MDR</i> 3 gene cause progressive familial intrahepatic cholestasis

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736 citations

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735 citations