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The most common causes of MODY are related to mutations in MODY1, MODY2 and MODY3. Although individuals who carry MODY2 mutations have a mild form of the disease, those who carry MODY1 and MODY3 variants have a much more severe expression that is also associated with long-term complications that develop in persons with T1D and T2D.
 
There also has been a link between MODY3 and MODY5 because of their interaction in terms of gene expression.  However, it is now becoming clear that the metabolic phenotype of individuals with these two forms of the disease is actually quite different.  To date, little has been known about MODY5 other than its association with renal cysts. However, it now appears that MODY5 is more often characterized by hyperinsulinemia and dyslipidemia (and more closely related to insulin resistance) than MODY3.  Thus, knowledge about the underlying MODY defect is likely to lead to better management and an improved prognosis for individuals with the disease.
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