MODY (Maturity Onset Diabetes of the Young)

MODY (Maturity Onset Diabetes of the Young)

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 HNF1α-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.

People with MODY often have symptoms or lab results that are unusual for type 1 or type 2 diabetes. For example, MODY might be present in:

  • a “type 1” diabetes patient who has negative blood testing for autoantibodies, typically done at the time of diabetes diagnosis (antibodies typically tested include one or more of the following: GAD65, islet cell or ICA,  IA-2, insulin, ZnT8)
  • a “type 1” diabetes patient who generates a significant amount of insulin years beyond diagnosis (detectable blood levels of c-peptide, proinsulin, and/ or insulin)
  • a “type 2” diabetes patient who is normal weight or not significantly overweight and shows no signs of insulin resistance
  • a diabetes patient who is part of a family in which three or more consecutive generations have been diagnosed with diabetes
  • a diabetes patient with stable, mildly elevated blood sugars, often found incidentally during a check-upMODY presents with symptoms that often veer from the norm for type 1 or type 2 diabetes.

Do you you believe that you, your child, or a family member might have MODY?

The Kovler Diabetes Center has a MODY Registry that helps researchers study MODY and uncover diabetes patterns in families. Learn more about enrolling in our MODY Registry.