MODY diabetes is Maturity onset diabetes of the young. It is rare, accounting for just 1% - 2% of all diabetes. MODY patients displayed a familial form of noninsulin – depenedent diabetes. Maturity-onset of diabetes of the young, or MODY, is a form of diabetes that is caused by mutations in a number of different genes. MODY is a form of monogenic diabetes. Each different mutated gene causes a slightly different type of diabetes. The most common forms are HNF1á-MODY (MODY3) and GCK-MODY (MODY2), due to mutations in the HNF1A and GCK genes, respectively. MODY is typically diagnosed in late childhood, adolescence, or early adulthood.  However, it has been known to develop in adults as late as their 50s.  Many people with MODY are misdiagnosed as having type 1 or type 2 diabetes. However, a diagnosis of MODY could change the course of treatment and could help to identify other family members with MODY. MODY forms of diabetes are caused by at least nine different genes, some related to each other in function and some not. The commercially available MODY gene test suite only tests for six of these genes. MODY genes have one major thing in common—they are “monogenic” which means that you only need to inherit one copy of the gene to display the disorder that the gene causes. MODY forms of diabetes were long believed to affect around 2% of all people diagnosed with both type 1 and type 2 diabetes. However, a study of 586 children diagnosed with Type 1 diabetes found that a full 8% of them were actually carrying one of the three most common MODY genes. It is likely that a similar number of people diagnosed with Type 2 may also have one of these genetic forms of diabetes, too.

Keywords: MODY - Maturity onset diabetes of the young; HNF -  Hepatocyte Nuclear Factor; IPF - Insulin Promoter Factor; BLK - B-lymphocyte tyrosin kinase.