What is Maturity Onset Diabetes of the Young?

Maturity Onset Diabetes of the Young (MODY) is a type of diabetes. Diabetes is a type of metabolic disorder in which the levels of blood sugar (glucose) are elevated. Diabetes is caused due to an absolute or relative deficiency of insulin (lack of insulin or the resistance of the cells to insulin). Insulin is a hormone secreted by the pancreas, which allows the cells in the body to use glucose in the blood for energy. This keeps the blood glucose under control. Lack of insulin (absolute or resistance of the cells to insulin) prevents the right amount of glucose from entering the cells. This causes glucose to build up in your blood resulting in hyperglycemia (high levels of glucose in the blood).

MODY affects one to two per cent of people with diabetes, but it often goes unrecognised or undiagnosed in many patients and the person may be treated as Type 1 or Type 2 diabetes by their doctors. The main features of MODY are:

• Onset of diabetes occurs mostly before the age of 25 years.
• Strong family history of diabetes (it tends to run in the family (passed on from an affected parent in one generation to the children). If a parent has MODY, there is 50% chance of passing on the defective gene to the child. This type of inheritance is called autosomal dominant inheritance.
• Many patients with MODY do not need insulin and can be treated with diabetes pills or meal planning.
• People with MODY fail to secrete enough insulin unlike people with type 2 diabetes who frequently produce lots of insulin, but their cells do not respond to insulin (insulin resistance). Defect of insulin secretion in MODY is caused due to the primary defect in the function of beta cells of the pancreas.

MODY is caused due to monogenetic defects in beta-cell function of the pancreas (these cells secrete insulin). There are several forms of Maturity Onset Diabetes of the Young: MODY 1 to 6. Each is caused by a single gene not working correctly unlike Type 2 diabetes in which minor problems in several genes at one time causes diabetes. MODY-2 and MODY-3 are common variants of MODY.

Currently, there is a great interest in MODY as it has a genetic basis. Scientists are trying to understand the gene defect and the mechanisms causing diabetes as it can provide insights into potential treatments for diabetes.

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