Liver-restricted deletion of the biliary atresia candidate gene Pkd1l1 causes bile duct dysmorphogenesis and ciliopathy

A recent multicenter genetic exploration of the biliary atresia splenic malformation (BASM) syndrome identified mutations in the ciliary gene PKD1L1 as candidate etiologic contributors. We hypothesized that deletion of Pkd1l1 in hepatoblasts would provide a mouse model of the developmental cholangiopathy of BA. We then performed gene expression profiling analysis using data obtained from total RNA-seq, isolated from the livers of Pkd1l1Fl/Fl (Fl/Fl) and Pkd1l1Fl/Fl x AFP-CRE mice (Pkd1l1ΔExon8/ΔExon8 Liver knockout ; LKO) at 7 weeks. Comparative gene expression profiling analysis of RNA-seq data for total RNA extracted from Fl/Fl and LKO livers.