HNF1B and polycystic kidney disease: Similarly, mutations in HNF-1β gene are seen in the autosomal dominant disorder MODY5 (maturity-onset diabetes mellitus of the young, type 5)[9] and MODY5 patients present type 2 diabetes and develop congenital kidney abnormalities including simple cysts, polycystic kidneys, cystic dysplasia and glomerulocystic kidney disease.