Maturity Onset Diabetes of the Young MODY

MODY are a group of monogenic disorders inherited in an autosomal dominant pattern. MODY is characterized by early onset (usually before the age of 25 years) of T2D fi cell dysfunction and there being a family history (at least two generations) of early onset diabetes. The defect is in insulin secretion due to mutations in the glucokinase and fi cell transcription factor genes (Table 2). Hepatocyte nuclear factors (HNF) 1a, 1fi, and 4a, insulin promoter factor (IPF1), and neurogenic differentiation^ (NEUROD1) play an important role in the normal development and function of the fi cells of the pancreas. In the UK mutations in HNF1a is the commonest cause of MODY accounting for 63% of cases, followed by mutations in the glucokinase gene (20% of cases). The clinical presentation and progression of diabetes is

Table 2 Maturity onset diabetes of the young

MODY Gene Chromosome MODY

subgroup frequency

Table 2 Maturity onset diabetes of the young

MODY Gene Chromosome MODY

subgroup frequency

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