Mutations in the PKHD1 gene, which encodes fibrocystin, cause autosomal recessive
polycystic kidney disease (ARPKD). Unfortunately, the lack of specific antibodies to the
mouse protein impairs the study of splicing, post-translational processing, shedding, and
temporal and spatial expression of endogenous fibrocystin at the cellular and subcellular
level. Here, we report using a knock-in strategy to generate a null Pkhd1 strain and a strain
that expresses fibrocystin along with two SV5-Pk epitope tags engineered in-frame into the …