- Tools
- Downloads
- Links
- Community
- About
- Help
FB2026_01
,
released March 12, 2026
Maturity-onset diabetes of the young, type 2 (MODY2), an autosomal dominant disorder, is one of multiple diseases affecting glucose metabolism for which the human GCK (glucokinase) gene is implicated. For information about fly models, see 'glucose metabolism disease, GCK-related' (FBhh0001313).
The human GCK gene has not been introduced into flies.
[updated Feb. 2021 by FlyBase; FBrf0222196]
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]
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, TYPE 2; MODY2](https://omim.org/entry/125851)
[GLUCOKINASE; GCK](https://omim.org/entry/138079)
Glucokinase-related MODY2 is a common form of the disorder, especially in children with mild hyperglycemia and in women with gestational diabetes and a family history of diabetes (Fajans et al., 2001; pubmed:11575290). Less than 50% of the carriers have overt diabetes; many of those who do are obese or elderly. Two percent of MODY2 patients require insulin therapy. [from MIM:125851; 2021.02.06]
Maturity-onset diabetes of the young type 2 (MODY2) is caused by heterozygous mutation in the GCK gene. [from MIM:125851; 2021.02.06]
GCK encodes glucokinase, a member of the hexokinase family of proteins. Hexokinases phosphorylate glucose to produce glucose-6-phosphate, the first step in most glucose metabolism pathways. In contrast to other forms of hexokinase, this enzyme is not inhibited by its product glucose-6-phosphate but remains active while glucose is abundant. Compared to other hexokinases, has a weak affinity for D-glucose, and is effective only when glucose is abundant. [Gene Cards, GCK; 210206]