title: [Monogenic forms of diabetes mellitus].
abstract: Through the years the evolution of the concepts in the different classifications of Diabetes Mellitus keep a close relationship with the advance of the scientific knowledge of this matter. In this way the last classification adopted in 1995 by the American Association of Diabetes and the World Health Organization has an etiologic basis, but in some aspects is already obsolete. With the new knowledges this classification should be modified including a group dedicated to monogenic diabetes. In this group should be considered diabetes MODY 1 to 6, constituted by the forms expressed by mutations of transcription factors and the glucokinase enzyme. There are other patients named MODY X, because the responsible genes of the diseases have not been identified yet. Also in this section might be included the mutations of the Kir6.2 subunit of the K+ channel recently described in the beta cell. It is possible that in a near future other forms will be described, which will contribute to a better understanding of the monogenic diabetes.
authors.forename: Enrique Blazquez
authors.initials: EB
authors.lastname: Fernández
mesh_terms: D000328:Adult; D000367:Age Factors; D000818:Animals; D003921:Diabetes Mellitus, Experimental; D003922:Diabetes Mellitus, Type 1 / Q000188:drug therapy / Q000235:genetics*; D003924:Diabetes Mellitus, Type 2 / Q000145:classification / Q000235:genetics*; D005941:Glucokinase / Q000235:genetics; D051557:Hepatocyte Nuclear Factor 4; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000008:administration & dosage / Q000378:metabolism / Q000627:therapeutic use; D000078790:Insulin Secretion; D007515:Islets of Langerhans; D051379:Mice; D009154:Mutation; D015221:Potassium Channels / Q000235:genetics; D013997:Time Factors; D014157:Transcription Factors / Q000235:genetics; D014944:World Health Organization
chemical_list: C496592:HNF4A protein, human; D051557:Hepatocyte Nuclear Factor 4; D007328:Insulin; D015221:Potassium Channels; D014157:Transcription Factors; D005941:Glucokinase