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Mutations in either the <jats:italic><jats:styled-content style=\"fixed-case\">PKD1<\/jats:styled-content><\/jats:italic> or <jats:italic><jats:styled-content style=\"fixed-case\">PKD2<\/jats:styled-content><\/jats:italic> genes, which encode polycystin 1 and polycystin 2, are the underlying cause of <jats:styled-content style=\"fixed-case\">ADPKD<\/jats:styled-content>. Progressive cyst formation and renal enlargement lead to renal insufficiency in these patients, which need to be managed by lifelong dialysis or renal transplantation. While characteristic features of <jats:styled-content style=\"fixed-case\">PKD<\/jats:styled-content> are abnormalities in epithelial cell proliferation, fluid secretion, extracellular matrix and differentiation, the molecular mechanisms underlying these events are not understood. 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