Maturity-Onset Diabetes of the Young (MODY) is the classical example of monogenic disease with autosomal dominant inheritance. MODY represents a group of clinically and genetically heterogeneous familial disorders with clinical appearance similar to non-insulin-dependent type 2 (5,6). MODY is characterized by autosomal dominant inheritance and early-onset of diabetes, typically but not exclusively before the age of 25 (7). There are at least six different forms of MODY, each resulting from heterozygous mutations in six different genes encoding the key regulator of the glycolytic pathway the glucokinase gene (GCK/MODY2) (8) and transcription factors genes hepatic nuclear factor (HNF)-4a (MODY1) (9), HNF-1a (MODY3) (10), HNF-1P (MODY5) (11), insulin promoter factor (IPF)-1 (MODY4) (12) and NEURO D1/P2 (MODY6) (13). MODY2 and MODY3 have the highest prevalence in most populations (Table 1).
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