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Rev Med Liege. 2005 May-Jun;60(5-6):439-41.

[MODY types of diabetes mellitus].

[Article in French]

Author information

  • jacques.gerard@endocrinogerard.be

Abstract

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.

PMID:
16035308
[PubMed - indexed for MEDLINE]
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