Biochemical experiments have shown that PC1 and PC2 form a receptor-ion channel complex (Hanaoka et al., 2000; Yu et al., 2009) leading ADPKD to be classified among channelopathies, a heterogeneous group of disorders caused by the dysfunction of ion channels expressed in cellular membranes and many intracellular organelles. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.