maturity onset diabetes of the young

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maturity onset diabetes of the young2016-12-06T20:21:27+00:00

maturity onset diabetes of the young 


The term maturity onset diabetes of the young includes any of a number (13 at last count, but most commonly MODY types 2 and 3) of dominantly inherited, monogenic defects of insulin secretion which may develop at any age. 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 guilty gene have a 95% chance of developing MODY during their lifetimes. 

Diagnosing MODY is important because some forms don’t 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         Genes        OMIM number

• MODY 1    HNF4A       OMIM:125850

• MODY 2    GCK           OMIM:125851  

• MODY 3    HNF1A       OMIM:600496

• MODY 4    PDX1         OMIM:606392

• MODY 5    HNF1B       OMIM:137920

• MODY 6    NEUROD1 OMIM:606394

• MODY 7    KLF11        OMIM:610508

• MODY 8    CEL            OMIM:609812

• MODY 9    PAX4          OMIM:612225

• MODY 10  INS             OMIM:613370

• MODY 11  BLK            OMIM:613375

• MODY 12  There is no MODY 12 in the OMIM database

• MODY 13  KCNJ11     OMIM:616329

• MODY 14  APPL1       OMIM:616511


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