Previously, it had been shown that complete loss of function of Biccaudal C (BicC), the gene which is mutated in both the bpk mouse model leading to an ARPKD phenotype and the jcpk mouse model leading to an ADPKD phenotype [107,108], resulted in LR-axis defects by disrupting the planar alignment of motile cilia required for cilia-driven fluid flow. Here, BTK is linked to autosomal recessive polycystic kidney disease.