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.