HNF1B (Hepatocyte Nuclear Factor 1 beta) is a homeobox transcription factor that functions in nephron development and the development of the embryonic pancreas. Mutations in this gene result in developmental kidney diseases, including renal cysts, single kidneys and renal hypoplasia. It has also been associated with early onset diabetes, pancreatic hypoplasia, genital tract malformations, abnormal liver function and early onset gout. It is expressed in renal tubules, the gallbladder, liver, the pancreas, and epithelia of the gastrointestinal tract, and is nuclear in localization. References: Clissold 2015 Nature Reviews Nephrology 11:102; Yu Cell 2015 Biosci 5:58