  Single gene mutations have been implicated in the pathogenesis of a form of
diabetes mellitus (DM) known as the maturity-onset diabetes of the young
(MODY). However, there are diverse opinions on the suspect genes and
pathophysiology, necessitating the need to review and communicate the genes to
raise public awareness. We used the Google search engine to retrieve relevant
information from reputable sources such as PubMed and Google Scholar. We
identified 14 classified MODY genes as well as three new and unclassified genes
linked with MODY. These genes are fundamentally embedded in the beta cells, the
most common of which are HNF1A, HNF4A, HNF1B, and GCK genes. Mutations in these
genes cause beta-cell dysfunction, resulting in decreased insulin production
and hyperglycemia. MODY genes have distinct mechanisms of action and phenotypic
presentations compared with type 1 and type 2 DM and other forms of DM.
Healthcare professionals are therefore advised to formulate drugs and treatment
based on the causal genes rather than the current generalized treatment for all
types of DM. This will increase the effectiveness of diabetes drugs and
treatment and reduce the burden of the disease.
