Skip to main content
. Author manuscript; available in PMC: 2013 Jul 29.
Published in final edited form as: Clin Pharmacol Ther. 2012 Sep 5;92(5):599–612. doi: 10.1038/clpt.2012.79

Figure 2. Schematic representation of progressive familial intrahepatic cholestasis (PFIC) type 2.<.

Figure 2

br>Left panel: Under normal conditions BSEP transports bile acids into the bile. Right panel: Mutation of the ABCB11/BSEP gene results in proteasomal degradation and/or expression of a protein with low/no function, leading to reduced transport of bile acids into bile and consequently accumulation of deleterious bile acids in the hepatocyte. The broken lines represent minimal or no transport. ER, endoplasmatic reticulum.