DZIP1L is located at the basal body of the primary cilium, and impaired function of DZIP1L is associated with a defect in the ciliary trafficking of the two major proteins for autosomal dominant polycystic kidney disease (ADPKD), polycystin-1 and polycystin-2. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.