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Structural and biochemical aspects of keratan sulphate in the cornea

Quantock, Andrew James, Young, Robert David and Akama, Tomoya O. 2010. Structural and biochemical aspects of keratan sulphate in the cornea. Cellular and Molecular Life Sciences 67 (6) , pp. 891-906. 10.1007/s00018-009-0228-7

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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.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Optometry and Vision Sciences
Subjects: R Medicine > RE Ophthalmology
Uncontrolled Keywords: Keratan sulphate - Proteoglycan - Glycosaminoglycan - Cornea - Collagen
Publisher: Springer Verlag
ISSN: 1420-682X
Last Modified: 20 Oct 2017 21:29
URI: http://orca.cf.ac.uk/id/eprint/18889

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