Mutations in the human genes encoding the tissue-specific transcription factors hepatocyte nuclear factor (HNF)1 , HNF1 and HNF4 are responsible for maturity onset diabetes of the young (MODY), a monogenic dominant inherited form of diabetes mellitus characterized by defective insulin secretion of the pancreatic -cells. In addition, the mutated HNF1 gene causes defective development of the kidney and genital malformation. This review summarizes the main features of these transcription factors and discusses potential events leading to the specific disease phenotypes.

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