GCK-MODY in the US Monogenic Diabetes Registry: Description of 27 unpublished variants

Diabetes Res Clin Pract. 2019 May:151:231-236. doi: 10.1016/j.diabres.2019.04.017. Epub 2019 May 4.

Abstract

We report on 134 unique GCK variants in 217 families, including 27 unpublished variants, identified in the US Monogenic Diabetes Registry in the last decade. Using ACMG guidelines, 26% were pathogenic, 56% likely pathogenic and 18% were of uncertain significance. Those with pathogenic variants had clinical features consistent with GCK-MODY.

Keywords: Glucokinase; MODY; Monogenic diabetes; Next-generation sequencing; Pathogenic variants.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Diabetes Mellitus, Type 2 / diagnosis*
  • Diabetes Mellitus, Type 2 / epidemiology*
  • Diabetes Mellitus, Type 2 / pathology
  • Female
  • Glucokinase / genetics*
  • Humans
  • Male
  • Registries
  • United States
  • Young Adult

Substances

  • Glucokinase

Supplementary concepts

  • Mason-Type Diabetes