In humans, mutations in the HNF-1β gene are associated with a number of diseases associated with defects in kidney development and a complex syndrome known as renal cysts and diabetes (RCAD), characterized by multiple abnormalities of the kidney, male and female genital tract, and by early-onset diabetes, pancreatic hypoplasia, and liver dysfunction [11,12]. Here, HNF1B is linked to renal cysts and diabetes syndrome.