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Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis

✍ Scribed by Peter L.M. Jansen*; Sandra S. Strautnieks‡; Emmanuel Jacquemin§; Michelle Hadchouel§; Etienne M. Sokal∥; Guido J.E.J. Hooiveld*; Johan H. Koning*; Alie de Jager-Krikken*; Folkert Kuipers¶; Frans Stellaard¶; Charles M.A. Bijleveld¶; Annette Gouw#; Harry van Goor#; Richard J. Thompson‡; Michael Müller*


Book ID
118521884
Publisher
Elsevier Science
Year
1999
Tongue
English
Weight
567 KB
Volume
117
Category
Article
ISSN
0016-5085

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Degradation of the bile salt export pump
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The bile salt export pump (Bsep) represents the major bile salt transport system at the canalicular membrane of hepatocytes. When examined in model cell lines, genetic mutations in the BSEP gene impair its targeting and transport function, contributing to the pathogenesis of progressive familial int