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Genetic and Rare Diseases Information Center (GARD)

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Monogenic diabetes


* Not a rare disease
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The most common forms of diabetes, type 1 and type 2, are polygenic, meaning the risk of developing these forms of diabetes is related to multiple genes [1][2]. Environmental factors, such as obesity in the case of type 2 diabetes, also play a part in the development of polygenic forms of diabetes. Polygenic forms of diabetes often run in families. Doctors diagnose polygenic forms of diabetes by testing blood glucose in individuals with risk factors or symptoms of diabetes [1].

Some rare forms of diabetes result from mutations in a single gene and are called monogenic [1][2]. Monogenic forms of diabetes may account for about 1 to 5 percent of all cases of diabetes in young people [1]. In some cases of monogenic diabetes, the gene mutation is inherited; but in others, the gene mutation develops spontaneously [1][2]. Most mutations in monogenic diabetes reduce the body's ability to produce insulin, a protein produced in the pancreas that is essential for the body to use glucose for energy [1][2]. As a result, monogenic diabetes can easily be mistaken for type 1 diabetes [2].

Last updated: 9/23/2011


  1. Monogenic Forms of Diabetes Mellitus: Neonatal Diabetes Mellitus and Maturity-onset Diabetes of the Young. National Diabetes Information Clearinghouse (NDIC). March 2007; Accessed 8/14/2008.
  2. What is Monogenic Diabetes? . The University of Chicago Kovler Diabetes Center. Accessed 9/23/2011.
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  • The National Diabetes Information Clearinghouse (NDIC) was established in 1978 to increase knowledge and understanding about diabetes among patients, health care professionals, and the general public. Click on the link to view information on this topic. 

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