maturity onset diabetes mellitus of the young

maturity onset diabetes of the young

Any of a number  of dominantly inherited, monogenic defects of insulin secretion occurring at any age (most commonly types 2 and 3). MODYs do not include any forms of type-2 diabetes, and comprise 1–2% of those initially diagnosed with diabetes. Given that MODYs are autosomal dominant, those with the faulty gene have a 95% chance of developing MODY during their lifetimes. Diagnosing MODY is important as some forms do not need insulin and may do well with oral hypoglycaemic agents, and because other family members can be identified and their treatment modified if appropriate.

MODY susceptibility genes:
• MODY type 1—HNF4A 
• MODY type 2—GCK     
• MODY type 3—HNF1A
• MODY type 4—PDX1
• MODY type 5—HNF1B
• MODY type 6—NEUROD1
• MODY type 7—KLF11
• MODY type 8—CEL
• MODY type 9—PAX4
• MODY type 10—INS
• MODY type 11—BLK

maturity onset diabetes mellitus of the young

Molecular endocrinology A mild form of type 2 DM, with disease onset before age 25; MODY is linked to a DM susceptibility gene  possibly on 20q13 and a defect of the glucokinase gene on chromosome 7. See Type 2 DM.
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