Discovery and structural development of small molecules that enhance transport activity of bile salt export pump mutant associated with progressive familial intrahepatic cholestasis type 2

2012 ◽  
Vol 20 (9) ◽  
pp. 2940-2949 ◽  
Author(s):  
Takashi Misawa ◽  
Hisamitsu Hayashi ◽  
Yuichi Sugiyama ◽  
Yuichi Hashimoto
Keyword(s):  
Bile Salt ◽  
Small Molecules ◽  
Intrahepatic Cholestasis ◽  
Structural Development ◽  
Bile Salt Export Pump ◽  
Transport Activity ◽  
Progressive Familial Intrahepatic Cholestasis

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

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

1999 ◽  
Vol 117 (6) ◽  
pp. 1370-1379 ◽  
Author(s):  
Peter L.M. Jansen ◽  
Sandra S. Strautnieks ◽  
Emmanuel Jacquemin ◽  
Michelle Hadchouel ◽  
Etienne M. Sokal ◽  
...  
Keyword(s):  
Bile Salt ◽  
Intrahepatic Cholestasis ◽  
Bile Salt Export Pump ◽  
Progressive Familial Intrahepatic Cholestasis

scholarly journals Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II

Hepatology ◽  
2008 ◽  
Vol 48 (5) ◽  
pp. 1558-1569 ◽  
Author(s):  
Lin Wang ◽  
Huiping Dong ◽  
Carol J. Soroka ◽  
Ning Wei ◽  
James L. Boyer ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Bile Salt ◽  
Intrahepatic Cholestasis ◽  
Type Ii ◽  
Bile Salt Export Pump ◽  
Progressive Familial Intrahepatic Cholestasis
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