Renal malformations may be linked to mutations in the hepatocyte nuclear factor-1alpha (MODY3) gene.

M aturity-onset diabetes of the young (MODY) is an autosomaldominant form of disease characterized by -cell defects and early age of diagnosis. So far, six MODY genes have been identified (1,2). MODY is sometimes accompanied by extrapancreatic features such as developmental malformations and physiological and biochemical abnormalities (3). The most frequent MODY3 subtype is caused by mutations in the hepatocyte nuclear factor (HNF)-1 gene, a transcription factor expressed in pancreas, kidney, liver, and gut.