[MODY types of diabetes mellitus].
Maturity-onset diabetes of the young (MODY) is a monogenic subtype of diabetes mellitus characterized by a young onset of type 2 diabetes, some abnormalities of the beta-cell function and an autosomal dominant inheritance with high penetrance. MODY types represent less than 5% of all cases of type 2 diabetes. Six genetic mutations have been described, one of them affecting the glucokinase gene (MODY 2) and the others various transcription factors HNF-1alpha, HNF-4alpha, HNF-1beta, IPF-1 and NeuroD (MODY 1,3,4,5,6, respectively). These different underlying gene mutations are associated with different clinical forms of the disease. Among the two most frequent forms, MODY 2 (mutation of the glucokinase gene) has a benign clinical evolution whereas MODY 3 (mutation of HNF-1alpha gene) has a much more severe evolution. The recognition of the MODY diabetes is important in clinical practice and may lead to the discovery of new more specific molecular therapeutic targets.
Gérard
J
J
jacques.gerard@endocrinogerard.be
D017668:Age of Onset; D000368:Aged; D003924:Diabetes Mellitus, Type 2 / Q000209:etiology* / Q000235:genetics*; D020022:Genetic Predisposition to Disease; D005941:Glucokinase / Q000235:genetics*; D006801:Humans; D007515:Islets of Langerhans / Q000473:pathology; D008875:Middle Aged; D009154:Mutation; D014157:Transcription Factors / Q000235:genetics*
D014157:Transcription Factors; D005941:Glucokinase