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DCN gene
URL of this page: https://medlineplus.gov/genetics/gene/dcn/

DCN gene

decorin

Normal Function

The DCN gene provides instructions for making a protein called decorin. This protein is a component of the extracellular matrix, which is the intricate lattice of proteins and other molecules that forms in the spaces between cells. Decorin is found in the extracellular matrix of a variety of connective tissues, which support the body's joints and organs.

Decorin is involved in the organization of collagens. Collagens are proteins that strengthen and support connective tissues throughout the body. Collagens also play an important role in the cornea, which is the clear layer that covers the front of the eye. Bundles of collagen called fibrils must be strictly organized for the cornea to be transparent. Decorin helps ensure that these collagen fibrils are the right size and are regularly spaced.

Researchers have proposed several additional functions for decorin. This protein likely helps regulate the activity of multiple growth factors, which are proteins that control a range of processes that are important for cell growth and survival. Studies suggest that decorin also plays a role in the attachment of cells to one another (cell adhesion), the formation of new blood vessels (angiogenesis), and the regulation of inflammation. Decorin may also act as a tumor suppressor, which means that it keeps cells from growing and dividing (proliferating) too fast or in an uncontrolled way.

Health Conditions Related to Genetic Changes

Congenital stromal corneal dystrophy

Several variants (also called mutations) in the DCN gene have been associated with congenital stromal corneal dystrophy. This condition is an inherited eye disorder that causes the corneas to become cloudy. The DCN gene variants that cause congenital stromal corneal dystrophy typically lead to the production of an abnormally short version of the decorin protein. This abnormal protein is thought to accumulate in the cornea. This causes the cornea to become cloudy, which can impair a person’s ability to see clearly (reduced visual acuity). The accumulation of abnormal decorin may also lead to the other eye abnormalities seen in people with congenital stromal corneal dystrophy.

More About This Health Condition

Other Names for This Gene

  • decorin proteoglycan
  • dermatan sulphate proteoglycans 2
  • DSPG2
  • PG40
  • PGII
  • PGS2
  • proteoglycan II
  • SLRR1B

Additional Information & Resources

Tests Listed in the Genetic Testing Registry

Scientific Articles on PubMed

Catalog of Genes and Diseases from OMIM

Gene and Variant Databases

References

  • Bredrup C, Knappskog PM, Majewski J, Rodahl E, Boman H. Congenital stromal dystrophy of the cornea caused by a mutation in the decorin gene. Invest Ophthalmol Vis Sci. 2005 Feb;46(2):420-6. doi: 10.1167/iovs.04-0804. Citation on PubMed
  • Bredrup C, Stang E, Bruland O, Palka BP, Young RD, Haavik J, Knappskog PM, Rodahl E. Decorin accumulation contributes to the stromal opacities found in congenital stromal corneal dystrophy. Invest Ophthalmol Vis Sci. 2010 Nov;51(11):5578-82. doi: 10.1167/iovs.09-4933. Epub 2010 May 19. Citation on PubMed
  • Danielson KG, Fazzio A, Cohen I, Cannizzaro LA, Eichstetter I, Iozzo RV. The human decorin gene: intron-exon organization, discovery of two alternatively spliced exons in the 5' untranslated region, and mapping of the gene to chromosome 12q23. Genomics. 1993 Jan;15(1):146-60. doi: 10.1006/geno.1993.1022. Citation on PubMed
  • Jarvinen TA, Prince S. Decorin: A Growth Factor Antagonist for Tumor Growth Inhibition. Biomed Res Int. 2015;2015:654765. doi: 10.1155/2015/654765. Epub 2015 Nov 30. Citation on PubMed
  • Rodahl E, Knappskog PM, Bredrup C, Boman H. Congenital Stromal Corneal Dystrophy. 2008 Nov 25 [updated 2018 Nov 29]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2025. Available from http://www.ncbi.nlm.nih.gov/books/NBK2690/ Citation on PubMed
  • Rodahl E, Van Ginderdeuren R, Knappskog PM, Bredrup C, Boman H. A second decorin frame shift mutation in a family with congenital stromal corneal dystrophy. Am J Ophthalmol. 2006 Sep;142(3):520-1. doi: 10.1016/j.ajo.2006.03.064. Citation on PubMed
  • Ruhland C, Schonherr E, Robenek H, Hansen U, Iozzo RV, Bruckner P, Seidler DG. The glycosaminoglycan chain of decorin plays an important role in collagen fibril formation at the early stages of fibrillogenesis. FEBS J. 2007 Aug;274(16):4246-55. doi: 10.1111/j.1742-4658.2007.05951.x. Epub 2007 Jul 25. Citation on PubMed
  • Zhang G, Chen S, Goldoni S, Calder BW, Simpson HC, Owens RT, McQuillan DJ, Young MF, Iozzo RV, Birk DE. Genetic evidence for the coordinated regulation of collagen fibrillogenesis in the cornea by decorin and biglycan. J Biol Chem. 2009 Mar 27;284(13):8888-97. doi: 10.1074/jbc.M806590200. Epub 2009 Jan 9. Citation on PubMed or Free article on PubMed Central
  • Zhang W, Ge Y, Cheng Q, Zhang Q, Fang L, Zheng J. Decorin is a pivotal effector in the extracellular matrix and tumour microenvironment. Oncotarget. 2018 Jan 3;9(4):5480-5491. doi: 10.18632/oncotarget.23869. eCollection 2018 Jan 12. Citation on PubMed

The information on this site should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.