Hepatocyte nuclear factor 1-alpha (HNF1A, TCF1) and hepatocyte nuclear factor 1-beta (HNF1B, TCF2) are homeodomain-containing transcription factors expressed in the pancreas, liver, kidney, and intestine, where they direct tissue-specific gene regulation [, ]. They play an essential role in the development and differentiation of pancreatic precursor cells [, ]. Mutations in HNF1beta and HNF1alpha cause autosomal dominant diabetes maturity-onset diabetes of the young (MODY), which is a monogenic form of non-insulin-dependent type 2 diabetes. Mutations in HNF1A are responsible for type 3 MODY (MODY3) [], whereas the HNF1B gene is linked to a rare case of type 5 MODY (MODY5) [].
