MODY is a monogenic form of diabetes with an autosomal dominant mode of inheritance:
Mutations in any one of several transcription factors or in the enzyme glucokinase lead to insufficient insulin release from pancreatic ß-cells, causing MODY.
Different subtypes of MODY are identified based on the mutated gene.
Originally, diagnosis of MODY was based on presence of non-ketotic hyperglycemia in adolescents or young adults in conjunction with a family history of diabetes.
