PMID: 9539292Apr 16, 1998Paper

Genetic, metabolic and clinical characteristics of maturity onset diabetes of the young

European Journal of Endocrinology
G Velho, P Froguel

Abstract

Maturity onset diabetes of the young (MODY) is a genetically and clinically heterogeneous subtype of non-insulin-dependent diabetes mellitus (NIDDM) characterised by early onset, autosomal dominant inheritance and a primary defect in insulin secretion. To date, three MODY genes have been identified on chromosomes 20q (MODY1/hepatic nuclear factor (HNF)-4alpha), 7p (MODY2/glucokinase) and 12q (MODY3/HNF-1alpha). Mutations in MODY2/glucokinase result in mild chronic hyperglycaemia as a result of reduced pancreatic beta-cell responsiveness to glucose, and decreased net accumulation of hepatic glycogen and increased hepatic gluconeogenesis after meals. In contrast, MODY1 and MODY3 are characterised by severe insulin secretory defects, and by major hyperglycaemia associated with microvascular complications. The role of the three known MODY genes in susceptibility to the more common late-onset NIDDM remain uncertain. Genetic studies seem to exclude a role as major susceptibility genes, but leave unresolved whether they may have a minor role in a polygenic context or an important role in particular populations.

Citations

Dec 30, 2006·Reviews in Endocrine & Metabolic Disorders·Daniel E Hale, Guadalupe Rupert
May 10, 2001·Trends in Biochemical Sciences·F RollandJ M Thevelein
May 14, 1999·Trends in Endocrinology and Metabolism : TEM·P Froguel, G Velho
Mar 12, 2004·Seminars in Neonatology : SN·Michel Polak, Julian Shield
May 18, 1999·Molecular Medicine Today·F Levine, G Leibowitz
Oct 31, 2001·Clinical Genetics·C P Busch, R A Hegele
May 17, 2006·International Journal of Obesity : Journal of the International Association for the Study of Obesity·R DefayA Fontbonne
Sep 5, 2002·Diabetic Medicine : a Journal of the British Diabetic Association·K R OwenA T Hattersley
Jun 16, 2005·Diabetes, Obesity & Metabolism·Fernando M A Giuffrida, André F Reis
Jun 5, 2011·Journal of Diabetes Investigation·Rochelle N NaylorLouis H Philipson
Mar 14, 2007·Orphanet Journal of Rare Diseases·Michel Polak, Hélène Cavé
Aug 29, 2000·Pediatric Research·D J Hill, B Duvillié
Dec 29, 2012·Genetics and Molecular Biology·Naieli BonattoRoberto F Artoni
Jul 20, 2005·Treatments in Endocrinology·Johanna K Wolford, Barbora Vozarova de Courten
Dec 1, 2001·Lancet·A Dornhorst
Feb 19, 2008·Best Practice & Research. Clinical Endocrinology & Metabolism·Isabelle FlechtnerMichel Polak
Oct 13, 2007·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·I FlechtnerM Polak
Sep 14, 2011·Pediatric Diabetes·J LudvigssonU Samuelsson
Dec 28, 1999·Endocrinology and Metabolism Clinics of North America·M W Haymond, A Sunehag
Jul 27, 2001·Diabetes·J Westerlund, P Bergsten
Sep 20, 2000·Journal of Molecular Biology·A A BoganF M Sladek
Jun 20, 2001·Acta Physiologica Scandinavica·C G Ostenson
Jul 27, 1999·Journal of Pediatric Endocrinology & Metabolism : JPEM·L GuazzarottiF Chiarelli
Jan 16, 2007·Journal of Pediatric Endocrinology & Metabolism : JPEM·Y HomanB Rami
Apr 2, 2002·Journal of Pediatric Endocrinology & Metabolism : JPEM·Constantine TsigosSotirios A Raptis
Jan 9, 2013·Arquivos brasileiros de endocrinologia e metabologia·Thais DellaMannaAndré Fernandes Reis
Dec 28, 1999·Endocrinology and Metabolism Clinics of North America·W E WinterD V House
Oct 2, 2001·American Journal of Medical Genetics·D C Wallace
Jan 26, 2002·British Medical Bulletin·A L Fowden, D J Hill
Aug 5, 1998·Proceedings of the National Academy of Sciences of the United States of America·R S StreeperR M O'Brien
Jan 16, 2004·International Journal of Obesity and Related Metabolic Disorders : Journal of the International Association for the Study of Obesity·M WabitschE Heinze
Dec 31, 2005·International Journal of Obesity : Journal of the International Association for the Study of Obesity·T Reinehr
Jun 7, 2002·The Journal of Clinical Endocrinology and Metabolism·R BarrioC Mustieles
Dec 3, 2009·American Journal of Physiology. Endocrinology and Metabolism·L van BürckN Herbach
Nov 2, 2014·Journal of Investigative Medicine : the Official Publication of the American Federation for Clinical Research·Clara Cieza-BorrellaRogelio González-Sarmiento

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