Although MODY is caused by a mutation in only one gene, there are 6 different types of MODY that are each caused by mutations in different genes. Thus, the mutation that causes MODY in one family may be different from the mutation that causes in another family.

This table summarize the MODY genes’ chromosomal locus, the protein encoded by the gene, the number of mutations identified, and the proportion of MODY cases caused by mutations in the gene. It is known that additional MODY genes have yet to be identified, as about 15% MODY families do not carry one of the confirmed mutations.

Each known MODY gene will be described separately in a subsequent slide.