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Genetics Home Reference: your guide to understanding genetic conditions
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DCN

Reviewed August 2009

What is the official name of the DCN gene?

The official name of this gene is “decorin.”

DCN is the gene's official symbol. The DCN gene is also known by other names, listed below.

What is the normal function of the DCN gene?

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, including skin, tendon, bone, and cartilage. Connective tissues support the body's joints and organs.

Decorin is involved in the organization of proteins called collagens. Collagens strengthen and support connective tissues throughout the body. Collagens also play an important role in the cornea, which is the clear outer covering of the eye. Bundles of collagen called fibrils must be strictly organized for the cornea to be transparent. Decorin ensures that these collagen fibrils are uniformly sized and regularly spaced.

Researchers have proposed several additional functions for decorin. This protein likely helps regulate cell growth and division, the attachment of cells to one another (cell adhesion), and the self-destruction of cells (apoptosis). Studies suggest that decorin plays a role in the formation of new blood vessels (angiogenesis), wound healing, bone development, inflammation, and preventing the growth of cancerous tumors. Decorin also regulates the activity of several growth factors, including transforming growth factor-beta (TGFβ). These growth factors control a diverse range of processes important for cell growth.

Does the DCN gene share characteristics with other genes?

The DCN gene belongs to a family of genes called proteoglycans (proteoglycans). It also belongs to a family of genes called small leucine-rich repeats (small leucine-rich repeats).

A gene family is a group of genes that share important characteristics. Classifying individual genes into families helps researchers describe how genes are related to each other. For more information, see What are gene families? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genefamilies) in the Handbook.

How are changes in the DCN gene related to health conditions?

congenital stromal corneal dystrophy - caused by mutations in the DCN gene

Several mutations in the DCN gene have been identified in families with congenital stromal corneal dystrophy. Each of the known mutations leads to the production of an abnormally short version of the decorin protein. This abnormal protein interferes with the organization of collagen fibrils in the cornea. As poorly arranged collagen fibrils accumulate, the cornea becomes cloudy. These corneal changes lead to a loss of sharp vision (reduced visual acuity) and other eye abnormalities related to visual impairment.

Where is the DCN gene located?

Cytogenetic Location: 12q21.33

Molecular Location on chromosome 12: base pairs 91,145,257 to 91,183,028

The DCN gene is located on the long (q) arm of chromosome 12 at position 21.33.

The DCN gene is located on the long (q) arm of chromosome 12 at position 21.33.

More precisely, the DCN gene is located from base pair 91,145,257 to base pair 91,183,028 on chromosome 12.

See How do geneticists indicate the location of a gene? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genelocation) in the Handbook.

Where can I find additional information about DCN?

You and your healthcare professional may find the following resources about DCN helpful.

You may also be interested in these resources, which are designed for genetics professionals and researchers.

What other names do people use for the DCN gene or gene products?

  • bone proteoglycan II
  • decorin proteoglycan
  • dermatan sulphate proteoglycans II
  • DSPG2
  • PG40
  • PGII
  • PGS2
  • PGS2_HUMAN
  • proteoglycan core protein
  • SLRR1B
  • small leucine-rich protein 1B

See How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What glossary definitions help with understanding DCN?

angiogenesis ; apoptosis ; cartilage ; cell ; cell adhesion ; collagen ; congenital ; cornea ; extracellular ; extracellular matrix ; gene ; growth factor ; inflammation ; leucine ; protein ; proteoglycan ; tendon ; visual acuity

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://www.ghr.nlm.nih.gov/glossary).

References

  • Bredrup C, Knappskog PM, Majewski J, Rødahl 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. (http://www.ncbi.nlm.nih.gov/pubmed/15671264?dopt=Abstract)
  • 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. (http://www.ncbi.nlm.nih.gov/pubmed/8432526?dopt=Abstract)
  • NCBI Gene (http://www.ncbi.nlm.nih.gov/gene/1634)
  • Rühland C, Schönherr 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. Epub 2007 Jul 25. (http://www.ncbi.nlm.nih.gov/pubmed/17651433?dopt=Abstract)
  • Rødahl 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. (http://www.ncbi.nlm.nih.gov/pubmed/16935612?dopt=Abstract)
  • 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. (http://www.ncbi.nlm.nih.gov/pubmed/19136671?dopt=Abstract)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: August 2009
Published: October 20, 2014