HomeGeneticsGeneDCN gene
Back to Gene
DCN gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

DCN gene

The DCN gene encodes the extracellular matrix protein decorin, which regulates collagen organization, growth‑factor activity, and tissue integrity.

Overview

DCN provides instructions for making a protein called decorin. Decorin is a component of the extracellular matrix, the lattice of proteins and other molecules that fills the spaces between cells. It is present in the extracellular matrix of many connective tissues that support joints and organs.

Decorin is involved in the organization of collagens. Collagens strengthen and support connective tissues throughout the body and are essential for corneal transparency. Bundles of collagen called fibrils must be strictly organized for the cornea to remain clear. Decorin helps ensure that these collagen fibrils are the correct size and are regularly spaced.

Researchers have proposed additional functions for decorin. The protein likely helps regulate the activity of multiple growth factors, influences cell adhesion, promotes angiogenesis, and modulates inflammation. Decorin may also act as a tumor suppressor, limiting uncontrolled cell proliferation.

Health Conditions Related to Genetic Changes

Congenital stromal corneal dystrophy

Several variants (mutations) in the DCN gene have been associated with congenital stromal corneal dystrophy, an inherited eye disorder that causes corneal clouding. The variants typically produce an abnormally short decorin protein that accumulates in the cornea, leading to reduced visual acuity and other eye abnormalities.

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

  • Tests of DCN

Scientific Articles on PubMed

  • PubMed

Catalog of Genes and Diseases from OMIM

  • DECORIN; DCN

Gene and Variant Databases

  • NCBI Gene
  • ClinVar

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.
  • 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.
  • 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.
  • Jarvinen TA, Prince S. Decorin: A Growth Factor Antagonist for Tumor Growth Inhibition. Biomed Res Int. 2015;2015:654765. doi: 10.1155/2015/654765.
  • Rodahl E, Knappskog PM, Bredrup C, Boman H. Congenital Stromal Corneal Dystrophy. 2008 Nov 25 [updated 2018 Nov 29]. In: GeneReviews® (Internet). Seattle (WA): University of Washington, Seattle; 1993-2026.
  • 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.
  • 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.
  • 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.
  • 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.

Genomic Location

The DCN gene is located on chromosome 12.

Frequently Asked Questions

Treatment for corneal clouding in congenital stromal corneal dystrophy often involves managing symptoms and improving vision. This may include the use of contact lenses or glasses to correct refractive errors, and in severe cases, corneal transplantation (keratoplasty) may be considered to replace the cloudy cornea with a clear donor cornea.
Congenital stromal corneal dystrophy is an inherited eye disorder. The inheritance pattern can vary, but if it's autosomal dominant, there's a 50% chance of an affected parent passing the gene mutation to each child. If it's autosomal recessive, both parents must be carriers for a child to be affected, with a 25% chance for each pregnancy. Genetic counseling can provide specific risk assessments.
Congenital stromal corneal dystrophy primarily causes corneal clouding, which means the clear front surface of the eye becomes opaque. This clouding can lead to reduced visual acuity, making vision blurry or hazy, and may cause other eye abnormalities from birth or early childhood.
Diagnosis typically involves a comprehensive eye examination by an ophthalmologist, who can observe the characteristic corneal clouding. Genetic testing can confirm the diagnosis by identifying specific mutations in the DCN gene, which are known to cause the condition.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

kontakt@hittavard.se

Ⓒ 2024. Alla rättigheter förbehålls av juxt ab

Sök
Om ossVillkor och policyVanliga frågorKontakta oss