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Diseases

Genetic and Rare Diseases Information Center (GARD)

Maturity-onset diabetes of the young


Other Names for this Disease
  • MODY
  • Mason type diabetes
Related Diseases
  • Maturity-onset diabetes of the young, type 1
  • Maturity-onset diabetes of the young, type 2
  • Maturity-onset diabetes of the young, type 3
  • Maturity-onset diabetes of the young, type 4
  • Maturity-onset diabetes of the young, type 5
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Overview

Maturity-onset diabetes of the young (MODY) is a form of diabetes that is characterized by an early onset diabetes.  MODY represents about 2% of all diabetes cases and is commonly misdiagnosed as type 1 or type 2 diabetes mellitus. It is due to a primary defect in pancreatic β-cell function.[1] There are several MODY subtypes with distinct genetic causes: MODY1, caused by mutations in the HNF4A geneMODY2, caused by mutations in GCK gene; MODY3 caused by mutations in the HNFA1 (the most common type); MODY4 caused by mutations in the PDX1 gene; MODY5 caused by mutations in the HNF1B gene; MODY6 caused by mutations in the NEUROD1 gene; MODY7 caused by mutations in the KLF11 gene; MODY8 caused by mutations in the CEL gene; MODY9 caused by mutations in the PAX4 gene; MODY10 caused by mutations in the INS gene; MODY11 caused by mutations in the BLK gene; MODY12 caused by mutations in the ABCC8 gene; MODY13  caused by heterozygous mutation in the KCNJ11 gene; and MODY14 caused by mutations in the APPL1 gene. It is inherited in an autosomal dominant pattern. Treatment depends on the subtype and may include sulfonylureas, insulin or diet and exercise.[1][2]
Last updated: 4/20/2016

References

  1. Kim S-H. Maturity-Onset Diabetes of the Young: What Do Clinicians Need to Know?. /Diabetes Metab J. December, 2015; 39(6):468–477. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4696982.
  2. Maturity-onset diabetes of the young. OMIM. 2016; http://www.omim.org/entry/606391.
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Basic Information

  • 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. 

In Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
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    Orphanet
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Maturity-onset diabetes of the young. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • MODY
  • Mason type diabetes
Related Diseases
  • Maturity-onset diabetes of the young, type 1
  • Maturity-onset diabetes of the young, type 2
  • Maturity-onset diabetes of the young, type 3
  • Maturity-onset diabetes of the young, type 4
  • Maturity-onset diabetes of the young, type 5
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.