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Cellular and Molecular Life Sciences

, Volume 67, Issue 6, pp 891–906 | Cite as

Structural and biochemical aspects of keratan sulphate in the cornea

  • Andrew J. QuantockEmail author
  • Robert D. Young
  • Tomoya O. Akama
Review

Abstract

Keratan sulphate (KS) is the predominant glycosaminoglycan (GAG) in the cornea of the eye, where it exists in proteoglycan (PG) form. KS-PGs have long been thought to play a pivotal role in the establishment and maintenance of the array of regularly-spaced and uniformly-thin collagen fibrils which make up the corneal stroma. This characteristic arrangement of fibrils allows light to pass through the cornea. Indeed, perturbations to the synthesis of KS-PG core proteins in genetically altered mice lead to structural matrix alterations and corneal opacification. Similarly, mutations in enzymes responsible for the sulphation of KS-GAG chains are causative for the inherited human disease, macular corneal dystrophy, which is manifested clinically by progressive corneal cloudiness starting in young adulthood.

Keywords

Keratan sulphate Proteoglycan Glycosaminoglycan Cornea Collagen 

Notes

Acknowledgments

Our research programmes are funded by BBSRC Project Grant BB/D001919/1 (UK), NIH/NEI Research Grant EY014620 (USA), MRC Programme Grant G0600755 (UK), and EPSRC Project Grant EP/F034970/1 (UK).

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Copyright information

© Birkhäuser Verlag, Basel/Switzerland 2009

Authors and Affiliations

  • Andrew J. Quantock
    • 1
    Email author
  • Robert D. Young
    • 1
  • Tomoya O. Akama
    • 2
    • 3
  1. 1.Structural Biophysics Group, School of Optometry and Vision SciencesCardiff UniversityCardiffWales, UK
  2. 2.Tumor Microenvironment ProgramBurnham Institute for Medical ResearchLa JollaUSA
  3. 3.Department of PharmacologyKansai Medical UniversityMoriguchiJapan

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