It typically causes end-stage renal failure (ESRF) in the 6th to 8th decades of life requiring renal replacement therapy such as dialysis or transplantation.1 About 85% of cases of ADPKD are caused by mutations in PKD1 with the remainder due to mutations in PKD2. 2PKD1 encodes polycystin-1 (PC-1), a large multi-membrane spanning glycoprotein of 4302 residues and mass >460 kDa. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.