Maturity-Onset Diabetes of the Young: An Overview with Focus on the Middle East

Curr Mol Med. 2017;17(8):549-562. doi: 10.2174/1566524018666180222121158.

Abstract

Today, there are more than 32 million patients with diabetes in the Middle East (ME), the number is expected to double by 2040. In contrast to Europe and North America and despite the high prevalence of diabetes in ME, epidemiological data about incident and types of diabetes in the region is very limited. Maturity-onset diabetes of the young (MODY) is a subtype of monogenic diabetes representing 2-5% of all diabetes patients. The disease is autosomal dominant mode of inheritance characterized by β-cell malfunction below the age of 35 years. To date, there are 15 different MODY forms that have been identified; each has a distinct genetic etiology. It is very common that MODY is misdiagnosed between with type 1 or type 2 diabetes. Hence, accurate genetic diagnosis is crucial to individualize the treatment and to improve glycaemia control. New technologies such as next-generation sequencing (NGS) offer an excellent alternative to screen and identify causative MODY mutations. In this review, we summarize the main clinical and genetic characteristics for each MODY form. In addition, we shed light on MODY studies that have been executed in the ME region.

Keywords: Maturity-onset diabetes of the young; Middle East; autosomal dominant mode; glycaemia; monogenic diabetes; next-generation sequencing..

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Diabetes Mellitus, Type 2 / diagnosis*
  • Diabetes Mellitus, Type 2 / epidemiology*
  • Diabetes Mellitus, Type 2 / genetics
  • Hepatocyte Nuclear Factor 4 / genetics*
  • High-Throughput Nucleotide Sequencing / methods*
  • Humans
  • Mass Screening*
  • Middle East / epidemiology
  • Mutation*
  • Prevalence

Substances

  • HNF4A protein, human
  • Hepatocyte Nuclear Factor 4

Supplementary concepts

  • Mason-Type Diabetes