We recently reported that the ABCB4 mutations p.G68R, p.G228R, p.D459H, and p.A934T, which we found in children with progressive familial intrahepatic cholestasis type 3 (PFIC3), resulted in ABCB4 protein being trapped in the ER [13,16]. This evidence concerns the gene ABCB4 and progressive familial intrahepatic cholestasis type 3.