What genes are associated with maturity onset diabetes of youth (MODY)?

Updated: Jul 13, 2021
  • Author: Romesh Khardori, MD, PhD, FACP; Chief Editor: George T Griffing, MD  more...
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Some forms of diabetes have a clear association with genetic defects. The syndrome historically known as maturity onset diabetes of youth (MODY), which is now understood to be a variety of defects in beta-cell function, accounts for 2-5% of individuals with type 2 diabetes who present at a young age and have mild disease. The trait is autosomal dominant and can be screened for through commercial laboratories.

To date, 11 MODY subtypes have been identified, involving mutations in the following genes [56, 57] :

  • HNF-4-alpha

  • Glucokinase gene

  • HNF-1-alpha

  • IPF-1

  • HNF-1-beta


  • KLF11 [58]

  • CEL [59]

  • PAX4 [60]

  • INS

  • BLK [61]

Most of the MODY subtypes are associated with diabetes only; however, MODY type 5 is known to be associated with renal cysts, [62] and MODY type 8 is associated with exocrine pancreatic dysfunction. [59]

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