Patients with autosomal dominant polycystic kidney disease (ADPKD) develop fluid-filled renal cysts that can precipitate kidney failure. PKD1 and PKD2, which are compromised in familial ADPKD, encode polycystin proteins that combine to form a cation channel in the sensory cilia of the renal epithelium. Miller and Portman show that polycystin homologues in C. elegans interact with a novel transmembrane protein CWP-5. CWP-5 mutation disrupts the formation of sensory cilia and may prevent toxic polycystin forms from reaching cilia.
2010
