Individuals with certain types of mutations may show a slightly raised blood sugar for life with mild or no symptoms of DM. 14 The phenotypic expressions of MODY depend on the causal gene. MODY genes disrupt insulin production processes, culminating in hyperglycemia, which with time, may damage organs such as eyes, kidneys, nerves, and blood vessels. 10 However, these symptoms are often unrecognized due to the low incidence of MODY, which is estimated to account for 1–5% of DM cases. 2, 8, 9 Some distinct characteristics of MODY include less significant weight gain, the absence of pancreatic autoantibodies, and lack of insulin resistance or elevated fasting glucose.
7 It was estimated that at least 90% of MODY diabetics are misdiagnosed as having T2DM due to lack of awareness on the differences between the two. Many physicians and researchers often considered and misdiagnosed MODY as a subset of T2DM. 5, 6 However, this classification may be confusing due to the similar pathophysiology of MODY and
#MODY DO FS 16 SERIES#
5, 6 The condition was later named MODY by Fajans Stefan after a series of studies.
3, 4 MODY was discovered by Robert Tattersall in 1974 as a distinct form of DM after discovering young non-insulin dependent diabetic individuals two-years post-diagnosis. 1, 2 The disease usually appears between the teen ages and early adulthood, < 25 years. Maturity-onset diabetes of the young (MODY) is a monogenic and non-autoimmune form of diabetes mellitus (DM) with characteristic pancreatic β-cell destruction and disrupted insulin biosynthesis. This will increase the effectiveness of diabetes drugs and treatment and reduce the burden of the disease. 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. MODY genes have distinct mechanisms of action and phenotypic presentations compared with type 1 and type 2 DM and other forms of DM. Mutations in these genes cause β-cell dysfunction, resulting in decreased insulin production and hyperglycemia. These genes are fundamentally embedded in the beta cells, the most common of which are HNF1A, HNF4A, HNF1B, and GCK genes. We identified 14 classified MODY genes as well as three new and unclassified genes linked with MODY. We used the Google search engine to retrieve relevant information from reputable sources such as PubMed and Google Scholar. However, there are diverse opinions on the suspect genes and pathophysiology, necessitating the need to review and communicate the genes to raise public awareness. 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).