Disrupted Wnt signalling components, including Wnt9b, Fat4, Vangl2 and Inversin are associated with polycystic kidney disease [[31], [32], [33], [34], [35], [36]] and frizzled-related-protein-4 is upregulated in human ADPKD and in ADPKD mouse models [37]. This evidence concerns the gene VANGL2 and autosomal dominant polycystic kidney disease.