Bile salt export pump-reactive antibodies form a polyclonal, multi-inhibitory response in antibody-induced bile salt export pump deficiency.

doi:10.1002/hep.28311

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Article

Bile salt export pump-reactive antibodies form a polyclonal, multi-inhibitory response in antibody-induced bile salt export pump deficiency.

Hepatology (Baltimore, Md.) - 1 Feb 2016

Stindt Jan, Kluge Stefanie, Dröge Carola, Keitel Verena, Stross Claudia, Baumann Ulrich, Brinkert Florian, Dhawan Anil, Engelmann Guido, Ganschow Rainer, Gerner Patrick, Grabhorn Enke, Knisely A S, Noli Khalid A, Pukite Ieva, Shepherd Ross W, Ueno Takehisa, Schmitt Lutz, Wiek Constanze, Hanenberg Helmut, Häussinger Dieter, Kubitz Ralf

Abstract excerpt

UNLABELLED: Progressive familial intrahepatic cholestasis type 2 (PFIC-2) is caused by mutations in ABCB11, encoding the bile salt export pump (BSEP). In 2009, we described a child with PFIC-2 who developed PFIC-like symptoms after orthotopic liver transplantation (OLT). BSEP-reactive antibodies were demonstrated to account for disease recurrence. Here, we characterize the nature of this antibody response in 7...

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PMID: 26516723
DOI: 10.1002/hep.28311

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ABCB11

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