Previously, research on HNF-1β has mainly focused on a variety of diseases caused by HNF-1β mutations during organ development, such as maturity-onset diabetes of the young type 5 (MODY5), renal cysts, neonatal diabetes, pancreatic hypoplasia, abnormal liver function, cholestasis, etc.14, 15. This evidence concerns the gene HNF1B and cystic kidney disease.