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Details for phenotype: Maturity onset diabetes of the young

EndoNet ID: ENP00486

Name

Maturity onset diabetes of the young

General information

This phenotype is pathologic
  • Maturity-onset diabetes of the young (MODY) is a clinically heterogeneous group of disorders characterized by nonketotic diabetes mellitus, an autosomal dominant mode of inheritance, an onset usually before the age of 25 years and frequently in childhood or adolescence, and a primary defect in the function of the beta cells of the pancreas. [1]
  • MODY can result from mutations in any one of at least six different genes. All these genes are expressed in beta cells, and mutation of any of them leads to beta-cell dysfunction and diabetes mellitus. [1]

Links to other resources

GO
OMIM 606391
MeSH term D003924
Disease database 8330

Phenotype triggers

  • insulin receptor
    in beta_cell_of_islet_of_Langerhans
    • Factors that affect insulin sensitivity, such as infection, puberty, pregnancy, and (in rare cases) obesity, may trigger the onset of diabetes and increase the severity of hyperglycemia in patients with MODY [1]
Reference