Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

MODY1 diabetes

MODY1 is an autosomal dominant early-onset type 2 diabetes caused by mutations of the HNF4A gene.


There are only some few cases reported throughout the word.

Test Strategy

The diagnostic procedures generally are the same for all types of MODY diabetes.


This gene is in the same signaling cascade as TCF1, the gene where defects are responsible for MODY3. Therefore the pathogenesis and clinical expression are similar.


MODY diabetes
MODY1 diabetes
MODY10 diabetes
MODY11 diabetes
MODY12 diabetes
MODY13 diabetes
MODY14 diabetes
MODY2 diabetes
MODY3 diabetes
MODY4 diabetes
MODY5 diabetes
MODY6 diabetes
MODY7 diabetes
MODY8 diabetes
MODY9 diabetes



Fajans SS et al. (2001) Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young.


Weng J et al. (2002) Screening for MODY mutations, GAD antibodies, and type 1 diabetes--associated HLA genotypes in women with gestational diabetes mellitus.


OMIM.ORG article

Omim 125850 [^]
Update: May 9, 2019