title: Structural and biochemical aspects of keratan sulphate in the cornea.
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.
authors.affiliation: Cardiff University, Wales, UK. QuantockAJ@cf.ac.uk
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Quantock
authors.forename: Robert D
authors.initials: RD
authors.lastname: Young
authors.forename: Tomoya O
authors.initials: TO
authors.lastname: Akama
mesh_terms: D000818:Animals; D003315:Cornea / Q000737:chemistry* / Q000378:metabolism*; D006801:Humans; D007632:Keratan Sulfate / Q000737:chemistry* / Q000378:metabolism* / Q000648:ultrastructure
chemical_list: D007632:Keratan Sulfate