There are several subtypes of MODY caused by mutations in at least 14 known genes (HNF4A, GCK, HNF1A, PDX1, HNF1B, NEUROD1, KLF11, CEL, PAX4, INS, BLK, ABCC8, KCNJ11, APPL1), which have in common a primary defect in insulin secretion associated with pancreatic beta cell dysfunction [5]. This evidence concerns the gene HNF4A and MODY.