of its function causes cystogenesis in human autosomal dominant polycystic kidney disease.
We have previously shown that recombinant human PC1 is cis-autoproteolytically cleaved at
the G protein-coupled receptor proteolytic site domain. To investigate the role of cleavage in
vivo, we generated by gene targeting a Pkd1 knockin mouse (Pkd1 V/V) that expresses
noncleavable PC1. The Pkd1 V/V mice show a hypomorphic phenotype, characterized by a …