Liver disease associated with hereditary defects of hepatobiliary transporters |
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| Authors: | Wendum Dominique |
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| Affiliation: | 1. Service d’anatomie et cytologie pathologiques, hôpital Saint-Antoine, AP–HP, 184, rue du faubourg-Saint-Antoine, 7551 Paris cedex 12, France;2. UPMC, UMR_S 938, université de Paris-06, 75005 Paris, France;3. Inserm, UMR_S 938, 75012 Paris, France |
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| Abstract: | The identification of biliary tranporters has enhanced our understanding of bile formation and some liver diseases. In this review, we first describe the main hepatobiliary transporters and their function. Then, some liver diseases related to mutations of biliary tranporters (FIC1/ATP8B1, BSEP/ABCB11, MDR3?/ABCB4 and MRP2/ABCC2) will be described with a focus on the pathological aspects. These diseases include progressive familial intrahepatic cholestasis (PFIC), benign recurrent intrahepatic cholestasis (BRIC), intrahepatic cholestasis of pregnancy, Dubin-Johnson's syndrome and low phospholipid associated cholelithiasis (LPAC). |
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| Keywords: | Transporteurs hépatobiliaires Cholestases MDR3/ABCB4 |
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