The most common types of MODY

  • HNF1-alpha. This gene causes about 70% of cases of MODY. It causes diabetes by lowering the amount of insulin made by the pancreas. Diabetes usually develops in adolescence or early twenties, and people with HNF1-alpha MODY generally don’t need to take insulin, they can be treated with small doses of a group of tablets called sulphonylureas (often used in Type 2 diabetes).
  • HNF4-alpha. This isn’t as common as the other forms of MODY. People who have inherited a change in this gene are likely to have had a birth weight of 9lb or more (around 4 kg). They may also have had a low blood sugar at, or soon after, birth which might have needed treatment. people with HNF4-alpha are generally treated with a sulphonylurea tablet but may progress on to needing insulin.
  • HNF1-beta. People with this type of MODY can have a variety of problems including renal cysts (cysts of the kidneys), uterine abnormalities and gout as well as diabetes. Often the renal cysts can be detected in the womb before a baby is born. The diabetes tends to develop later and insulin treatment is usually necessary, as well as following a healthy balanced diet and getting regular physical activity. HNF4-beta MODY also carries a risk of complications of diabetes.
  • Glucokinase. This gene helps the body to recognise how high the blood glucose level is in the body. When this gene isn’t working properly the body allows the level of blood glucose to be higher than it should be. Blood glucose levels in people with glucokinase MODY are typically only slightly higher than normal, generally between 5.5-8mmol/l. You don’t generally have symptoms of this type of MODY and so it’s often picked up through routine testing (eg during pregnancy). You don’t need any treatment for glucokinase MODY.

All types of MODY apart from glucokinase carry a risk of the long-term complications of diabetes so you should follow a healthy balanced diet and keep physically active as this helps to maintain good blood glucose and cholesterol levels which in turn reduce the risk of complications.



Important information if you have MODY

Testing for MODY



More Topics

Type 1 Diabetes Alström Syndrome
Type 2 Diabetes Neonatal diabetes
Wolfram Syndrome Preventing complications of Diabetes