Thus, maturity onset of diabetes of the young (MODY) is a rare form of diabetes with mutations often residing in exons encoding the functional domains of transcription factors such as hepatocyte nuclear factor hepatocyte nuclear factor 1 homeobox A (HNF1A) (9) and HNF4A (10), or of proteins involved in β cell glucose metabolism such as glucokinase (GCK) (11) (Table 1). Here, HNF4A is linked to diabetes mellitus.