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.
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.
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.[^]