Structural and biochemical aspects of keratan sulphate in the cornea

Quantock, Andrew James ORCID: https://orcid.org/0000-0002-2484-3120, Young, Robert David ORCID: https://orcid.org/0000-0002-8300-8002 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:	19 Oct 2022 08:44
URI:	https://orca.cardiff.ac.uk/id/eprint/18889

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