Polycystic Kidney, Autosomal RecessiveIsobutyratesReceptors, Cell SurfaceKidney Diseases, CysticPolycystic Kidney DiseasesTRPP Cation ChannelsHepatocyte Nuclear Factor 1-betaCiliaChromosomes, Human, Pair 6Biliary TractKidney TubulesMutationDNA Mutational AnalysisKidneyExonsBase SequenceMolecular Sequence DataMice, TransgenicRNA, MessengerReverse Transcriptase Polymerase Chain Reaction