49 ISSN 1758-1907 10.2217/DMT.13.59 © 2014 Future Medicine Ltd Diabetes Manage. (2014) 4(1), 49–61 Summary Monogenic diabetes is estimated to account for 0.5–1.2% of all diabetes cases and remains underdiagnosed. It consists of a variety of subtypes associated with mutations in more than 25 genes. The main groups of monogenic diabetes include maturity-onset diabetes of the young (MODY), permane...

Maturity-onset diabetes of the young (MODY) is a rare form of juvenile diabetes mellitus, defined by early onset, absence of ketosis, non-insulin-dependent diabetes and autosomal dominant inheritance. Advances in molecular genetic analysis have identified mutations accounting for different MODY subtypes, all of them associated with defects of insulin secretion. We present a case of a nine year-...

CONTEXT Maturity-onset diabetes of the young (MODY) is a monogenic form of diabetes mellitus characterized by autosomal dominant inheritance, early age of onset and a primary insulin secretion defect. Certain MODY gene sequence variants may be involved in polygenic forms of type 2 diabetes. OBJECTIVE We assessed the contribution of MODY genes to the etiology of type 2 early-onset diabetes in ...

Maturity-onset diabetes of the young (MODY) is a monogenic form of diabetes mellitus characterized by autosomal dominant inheritance, early age of onset, and pancreatic beta cell dysfunction. Heterozygous mutations in at least seven genes can cause MODY. In the present study we investigated the relative prevalence of GCK (glucokinase) and HNF1alpha (hepatocyte nuclear factor 1alpha) mutations, ...

Maturity-onset diabetes of the young (MODY) is a genetically diverse endocrine disorder autosomal dominant inheritance characterized by pancreatic β-cell dysfunction.Since clinical symptoms are so similar, it can be difficult to diagnose MODY accurately, important consider patients family history and any other genetic risk factors when making diagnosis.Glucokinase (GCK) was first gene identifi...

Maturity-onset diabetes of the young (MODY) is a clinically heterogeneous group of monogenic disorders characterized by autosomal dominant inheritance of young-onset, non-insulin-dependent diabetes. The genes involved are important in beta cell development, function and regulation and lead to disorders in glucose sensing and insulin secretion. Heterozygous GCK mutations cause impaired glucokina...

Maturity-onset diabetes of the young (MODY) is both a genetically and clinically heterozygous type of diabetes mellitus characterized by early onset (often before 25 yr of age) and absence of pancreatic β-cell autoimmunity markers (1). To date, mutations in several distinct genes have been implicated in MODY (1, 2). Among the different types of MODY, MODY5 is caused by mutations in the gene enc...

Diabetes mellitus with autosomal dominant inheritance, i.e., maturity-onset diabetes of the young (MODY), is a genetic form of diabetes mellitus. The MODY phenotype is associated with gene mutations leading to pancreatic β-cell dysfunction. Here, we present the clinical case of a 50-year-old proband with familial diabetes mellitus in five generations (proband, her mother, grandmother, great-gra...

Background: Non-ketotic diabetes mellitus is commonly seen in young Saudi Arabian adults. This could be either early onset of typical type 2 diabetes, T2DM or maturity-onset diabetes of the young, MODY. Consanguinity is also prevalent and clouds the inheritance pattern of the disease. Distinction between the two on clinical grounds alone is not always possible. Materials and Method: Diabetic pa...