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.