MODY is a monogenic disorder that results in a familial, young-onset non-insulin dependent form of diabetes, typically presenting in lean young adults before 25 years. Approximately 1% of diabetes has a monogenic cause but this is frequently misdiagnosed as type 1 or type 2 diabetes.

Maturity-onset diabetes of the young (MODY, monogenic diabetes), a range of rare but important forms of type 2 diabetes mellitus caused by a single autosomal dominant genetic defect. The two commonest forms are mutations of the HNF-1a gene (MODY 3), which often responds to treatment with "sulphonylurea drugs, and mutations of the glucokinase gene (MODY 2), causing a mild elevation of blood glucose levels usually responsive to dietary management.

Unit 1, 

Reference: McDonald TJ, Ellard S. Maturity onset diabetes of the young: identification and diagnosis. Ann Clin Biochem. 2013;50(Pt 5):403-415.

Reference: Oxford Concise Colour Medical Dictionary (7th Edition, Kindle Edition) Oxford University Press 2020 ISBN-13: 978-0198836629, p1835