== (A) The HEK 293 cells were transiently transfected with wt GFP-Bsep, PFIC II mutants and Gly
== (A) The HEK 293 cells were transiently transfected with wt GFP-Bsep, PFIC II mutants and Gly. the wild-type (wt) Bsep, and their ubiquitination is usually increased by the proteasome inhibitors. Overexpression of several E3 ubiquitin ligases, which are involved in ER-associated degradation (ERAD), lead to the decrease of both mutant and wt Bsep. Gene knockdown studies revealed that this ERAD E3s Rma1 and TEB4 contribute to the degradation of G238V, while HRD1 contributes to the degradation of a mutant lacking the lumenal glycosylation domain name (Gly). Furthermore we present evidence that G982R weakly associates with various components of the ER quality control system. These data together demonstrate that this PFIC II mutants and Gly are degraded by the ERAD pathway. Bile secretion is Ets1 usually mediated by several ATP…