Monogenic Diabetes

Reviewed by: HU Medical Review Board | Last reviewed: November 2022 | Last updated: December 2022

For people with diabetes, the body cannot effectively turn food into energy. Insulin, which comes from the pancreas, normally helps let sugar (glucose) from food into cells. The body can then use the sugar as energy.1

If someone has diabetes, they do not produce enough insulin to do this. Without insulin, the sugar stays in the bloodstream. This causes the health problems associated with diabetes, such as:1

  • Heart disease
  • Kidney disease
  • Vision loss

There are a few main kinds of diabetes that people can have. Most people know about type 1 and type 2 diabetes. These types are caused by issues in genes that are passed from parent to child. Multiple genes are responsible for the conditions.1

But there is another type of diabetes called "monogenic diabetes." This type is caused by a problem with only 1 gene. Monogenic diabetes makes up about 1 percent to 5 percent of cases among all people with diabetes. And diagnosing it correctly can be crucial for treatment.1,2

What causes monogenic diabetes, and what are the different types?

Monogenic diabetes is caused by a singular changed (mutated) gene. It usually comes from 1 parent who also has the disorder.1-3

There are 2 main types of monogenic diabetes. One type results in an issue with the cells responsible for producing insulin. This type causes the body not to produce enough (or any) insulin.2,3

The other type causes severe insulin resistance. Depending on when it starts, this type is called MODY (monogenic diabetes of the young) or neonatal diabetes.2-4

Each of the different types of monogenic diabetes comes from mutations in different genes. These genes include:2-4

  • HFN4A
  • HNF1A
  • GCK
  • IPF1
  • HNF1B
  • NEUROD1

How is monogenic diabetes different from other types of diabetes?

The change in a single gene that causes monogenic diabetes is what separates it from other types. This distinction is crucial for treatment. Overall, the symptoms are the same as other types of diabetes:2-4

Some things that make monogenic diabetes different from other types of diabetes include:2

  • Early diagnosis, within the first 6 months of life
  • Lacking the antibodies responsible for type 1 diabetes
  • Not being overweight
  • A family history of diabetes, particularly in a parent or sibling
  • Having a mild form of diabetes as a child, but not needing insulin
  • Having other genetic mutations

How is monogenic diabetes diagnosed?

The simplest and most certain way to diagnose monogenic diabetes is by genetic testing. This will show if there is a mutation in any of the genes known to be responsible for monogenic diabetes.2,3

Blood tests can also be helpful. But they may not separate monogenic diabetes from type 1 or type 2 diabetes. Your doctor will also consider the factors listed above that distinguish monogenic diabetes from types 1 and 2.2

How is monogenic diabetes treated?

Your treatment plan for monogenic diabetes depends on the underlying cause of your case. Sometimes insulin can help people with monogenic diabetes maintain a healthy blood sugar.

For other people, certain drugs called sulfonylureas are helpful. Your doctor and care team will determine what is best for you based on the cause and symptoms of your monogenic diabetes.2,3

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