Genetics of MODY - Maturity Onset Diabetes of the Young

Abstract
MODY is an autosomal dominant disorder of carbohydrate metabolism, which results in an impaired ability to produce insulin. MODY was first described in 1960s when diabetes mellitus was considered to have only two forms: type 1, juvenile, and type 2, adult-onset (maturity-onset). MODY is a distinct hereditary form of diabetes, clinically different from the two more common types. MODY is generally characterized by asymptomatic persistent hyperglycemia, and onset of diabetes before 25 years of age.

MODY is a condition that is often misdiagnosed. Further, the hereditary nature of this autosomal dominant disorder is frequently overlooked.

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• Types of MODY
• Genetics of MODY
• Prevalence of different types among various ethnic groups
• Genotype/phenotype correlation and penetrance of MODY

This review article analyzes numerous studies that investigated genetics and inheritance of MODY and related disorders, mostly between 1999 and 2006.

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