This report describes general characteristics of the group of diseases termed maturity-onset diabetes of the young (MODY). Maturity-onset diabetes of the young is defined as an autosomal dominant form of diabetes typically occurring before 25 years of age and caused by primary insulin secretion defects. Since there is increasing incidence of polygenic type 2 diabetes in childhood and adolescence, and patients with gene mutations characteristic of MODY often present with clinical diabetes later in life, substituting the term 'autosomal dominant type 2 diabetes' has been proposed. Up to 5% of all diabetes cases may be due to MODY. Noninsulin-dependent diabetes mellitus (see FBhh0000153) is distinct from MODY in that it is polygenic and characterized by variation due to gene-gene and gene-environment interactions.
Subtypes of MODY are listed in the table below, with a link to the human disease model report for the subtype studied in flies.
[updated Jul. 2017 by FlyBase; FBrf0222196]
Up to 5% of all diabetes cases may be due to MODY. (http://www.health.harvard.edu/diabetes/maturity-onset-diabetes-of-the-young-mody)
Maturity-onset diabetes of the young is defined as an autosomal dominant form of diabetes typically occurring before 25 years of age and caused by primary insulin secretion defects. Since there is increasing incidence of polygenic type 2 diabetes in childhood and adolescence, and patients with gene mutations characteristic of MODY often present with clinical diabetes later in life, substituting the term 'autosomal dominant type 2 diabetes' has been proposed. [from MIM:606391; 2017.01.23]
Noninsulin-dependent diabetes mellitus is distinct from MODY in that it is polygenic, characterized by gene-gene and gene-environment interactions with onset in adulthood, usually at age 40 to 60 but occasionally in adolescence if a person is obese. [from MIM:125853; 2017.01.23]
Eleven different types of MODY have been identified, caused by changes in 11 different genes. Treatment varies, depending on the type of MODY. (http://www.health.harvard.edu/diabetes/maturity-onset-diabetes-of-the-young-mody)