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
|
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: | 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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