ADPKD is the most common hereditary kidney disease, resulting from mutations in PKD1 (MIM#: 601313) and PKD2 (MIM#: 173910) genes, located in chromosomes 16p13.3 (in approximately 85% of cases) and 4q22.1 (in approximately 15% of cases), respectively. The gene discussed is PKD2; the disease is autosomal dominant polycystic kidney disease.