Allo-immunisation anti-BSEP (Bile Salt Export Pump) post-transplantation hépatique pour cholestase intrahépatique progressive familiale de type 2 (PFIC2) : une complication sous-estimée ?

2011 ◽  
Vol 18 (12) ◽  
pp. 1326
Author(s):  
E. Gonzales ◽  
B. Grosse ◽  
M.J. Redon ◽  
I. Friteau ◽  
A. Davit-Spraul ◽  
...  
Keyword(s):  
Bile Salt ◽  
Bile Salt Export Pump ◽  
Post Transplantation ◽  
Transplantation Hépatique

scholarly journals Type 2 inositol 1,4,5-trisphosphate receptor modulates bile salt export pump activity in rat hepatocytes

Hepatology ◽  
2011 ◽  
Vol 54 (5) ◽  
pp. 1790-1799 ◽  
Author(s):  
Emma A. Kruglov ◽  
Samir Gautam ◽  
Mateus T. Guerra ◽  
Michael H. Nathanson
Keyword(s):  
Bile Salt ◽  
Rat Hepatocytes ◽  
Bile Salt Export Pump ◽  
Inositol 1,4,5 Trisphosphate ◽  
Pump Activity ◽  
Trisphosphate Receptor

scholarly journals Retargeting of bile salt export pump and favorable outcome in children with progressive familial intrahepatic cholestasis type 2

Hepatology ◽  
2015 ◽  
Vol 62 (1) ◽  
pp. 198-206 ◽  
Author(s):  
Sharat Varma ◽  
Nicole Revencu ◽  
Xavier Stephenne ◽  
Isabelle Scheers ◽  
Françoise Smets ◽  
...  
Keyword(s):  
Bile Salt ◽  
Intrahepatic Cholestasis ◽  
Favorable Outcome ◽  
Bile Salt Export Pump ◽  
Progressive Familial Intrahepatic Cholestasis

scholarly journals The Bile Salt Export Pump: Molecular Structure, Study Models and Small-Molecule Drugs for the Treatment of Inherited BSEP Deficiencies

2021 ◽  
Vol 22 (2) ◽  
pp. 784
Author(s):  
Muhammad Imran Sohail ◽  
Yaprak Dönmez-Cakil ◽  
Dániel Szöllősi ◽  
Thomas Stockner ◽  
Peter Chiba
Keyword(s):  
Molecular Structure ◽  
Bile Salt ◽  
Intrahepatic Cholestasis ◽  
Treatment Options ◽  
Genetic Diseases ◽  
Structure Study ◽  
Current Treatment ◽  
Bile Salt Export Pump ◽  
Small Molecular Weight

The bile salt export pump (BSEP/ABCB11) is responsible for the transport of bile salts from hepatocytes into bile canaliculi. Malfunction of this transporter results in progressive familial intrahepatic cholestasis type 2 (PFIC2), benign recurrent intrahepatic cholestasis type 2 (BRIC2) and intrahepatic cholestasis of pregnancy (ICP). Over the past few years, several small molecular weight compounds have been identified, which hold the potential to treat these genetic diseases (chaperones and potentiators). As the treatment response is mutation-specific, genetic analysis of the patients and their families is required. Furthermore, some of the mutations are refractory to therapy, with the only remaining treatment option being liver transplantation. In this review, we will focus on the molecular structure of ABCB11, reported mutations involved in cholestasis and current treatment options for inherited BSEP deficiencies.

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