MATURITY-ONSET DIABETES OF THE YOUNG

MATURITY-ONSET DIABETES OF THE YOUNG
Presentation by:Stefan S. Fajans, MD
University of Michigan

Definition
* Type-2 diabetes mellitus in the young plus
* Autosomal dominant inheritance

Current Definition of MODY
* A heterogeneous disorder due to heterozygous monogenic mutations in one of at least 6 different genes
* Onset of diabetes early in life: childhood, adolescence, young adulthood
* Autosomal dominant inheritance
* Primary defect in insulin secretion

Heterozygous Gene Mutations Identified in MODY
Homozygous Mutations of MODY-Related Genes
MODY-Related Proteins
Distinguishing Clinical Characteristics of MODY and Type 2 Diabetes (DM2)
MODY1 (HNF-4a Mutation):
Tested and normal
Type 1 diabetes
PVD-A-G
Multiple offspring
MODY1 (HNF-4a Mutation):
Tested and normal
Type 2 diabetes
Phenotypic Expression and Natural History of MODY
MODY1 (HNF-4a Mutation):
Protocol for the Stepped Glucose Infusion Method to Determine Insulin Secretion Rate

MATURITY-ONSET DIABETES OF THE YOUNG.ppt