title: [From gene to disease; 'maturity-onset diabetes of the young' (MODY), monogenetic inheritable forms of diabetes mellitus].
abstract: Maturity-onset diabetes of the young (MODY) exhibits an autosomal dominant pattern of inheritance and can be divided in at least five subtypes (MODY 1 to 5), each subtype being caused by mutations in a specific gene. The unambiguous molecular diagnosis of the specific MODY subtype facilitates an early diagnosis of diabetes and can help to reduce the development of diabetic complications. Furthermore, MODY2 patients generally have a milder clinical course and fewer complications than MODY3 patients, who consequently require a more aggressive therapeutic approach.
authors.affiliation: Afd. Moleculaire Celbiologie, Leids Universitair Medisch Centrum, Postbus 9503, 2300 RA Leiden. j.a.maassen@lumc.nl
authors.forename: J A
authors.initials: JA
authors.lastname: Maassen
authors.forename: H H
authors.initials: HH
authors.lastname: Lemkes
authors.forename: M
authors.initials: M
authors.lastname: Losekoot
mesh_terms: D000328:Adult; D001786:Blood Glucose / Q000378:metabolism; D004252:DNA Mutational Analysis; D003924:Diabetes Mellitus, Type 2 / Q000097:blood / Q000145:classification / Q000453:epidemiology / Q000235:genetics*; D005820:Genetic Testing; D005941:Glucokinase / Q000172:deficiency / Q000235:genetics; D006801:Humans; D015994:Incidence; D007328:Insulin / Q000097:blood / Q000378:metabolism*; D007515:Islets of Langerhans / Q000378:metabolism; D009154:Mutation / Q000235:genetics*; D009426:Netherlands / Q000453:epidemiology; D012720:Severity of Illness Index
chemical_list: D001786:Blood Glucose; D007328:Insulin; D005941:Glucokinase