J Korean Diabetes.  2017 Sep;18(3):169-176. 10.4093/jkd.2017.18.3.169.

Genetic Diseases Associated with Diabetes Mellitus

  • 1Division of Endocrinology and Metabolism, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea. jhnoh@paik.ac.kr


Whereas most people with diabetes mellitus have type 1 or type 2 diabetes, there are other inherited forms of diabetes, including single-gene forms of diabetes and rare genetic syndromes. Monogenic forms of pancreatic beta cell dysfunction include maturity-onset diabetes of the young (MODY) and neonatal diabetes, with MODY being the most common form of inherited diabetes. Mitochondrial diabetes and monogenic severe insulin resistance are also inherited forms of diabetes. In addition, more than 100 genetic diseases are known to be associated with diabetes mellitus. Diagnosis of inherited diabetes has important implications for patients, allowing personalized management and screening of their relatives. This review briefly presents genetic diseases associated with diabetes mellitus.


Diabetes mellitus; Genetic diseases; inborn; Single-gene diabetes disorder

MeSH Terms

Diabetes Mellitus*
Diabetes Mellitus, Type 2
Insulin Resistance
Insulin-Secreting Cells
Mass Screening



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