Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threaten hereditary disease, caused by mutations of either PKD1 or PKD2 which respectively encode polycystin-1 (PC1) and polycystin-2 (PC2) [1].It affects 1 : 400 to 1 : 1000 live births and accounts for up to 10% of all patients on renal replacement therapy[2], [3]. Here, PKD1 is linked to autosomal dominant polycystic kidney disease.