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Macular corneal dystrophy type II:Multiple studies on a cornea with low levels of sulphated keratan sulphate

Quantock, A J and Fullwood, N J and Thonar, E J M A and Waltman, S R and Capel, M S and Ito, M and Verity, S M and Schanzlin, D J (1997) Macular corneal dystrophy type II:Multiple studies on a cornea with low levels of sulphated keratan sulphate. RNIB Eye Contact, 11. pp. 57-67. ISSN 0967-8719

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Abstract

We investigated an individual macular corneal dystrophy (MCD) type II cornea from a 42-year-old woman with markedly reduced antigenic keratan sulphate levels, A characteristic 4.6 Angstrom X-ray reflection was evident, and the mid-stroma contained 30% less sulphur than normal. Close packing of collagen was restricted to the superficial stroma, Abnormally large proteoglycan filaments were noted throughout the extracellular matrix and Descemet's membrane's posterior non-banded zone, but not its anterior banded zone. Small, collagen-associated stromal proteoglycans were susceptible to digestion with chondroitinase ABC, but not keratanase I or N-glycanase. On occasion, collagen fibrils ranged in size from 20 mm to 58 nm, with preferential diameters of 34 nm and 42 nm, Corneal guttae were evident, as were numerous endothelial inclusions, most probably due to intracellular fibrillo-granular vacuoles similar to those found in the stroma. The endothelium expressed reduced anti-keratan sulphate labelling.

Item Type: Article
Journal or Publication Title: RNIB Eye Contact
Uncontrolled Keywords: cornea ; macular corneal dystrophy ; proteoglycans ; glycosaminoglycans ; SYNCHROTRON X-RAY ; COLLAGEN FIBRILS ; SULFATE PROTEOGLYCAN ; ELECTRON-MICROSCOPY ; DIFFRACTION ; STROMA ; GLYCOSAMINOGLYCANS ; HETEROGENEITY ; THICKNESS ; CULTURES
Subjects: UNSPECIFIED
Departments: Faculty of Health and Medicine > Biomedical & Life Sciences
ID Code: 51150
Deposited By: ep_importer_pure
Deposited On: 18 Nov 2011 10:51
Refereed?: Yes
Published?: Published
Last Modified: 26 Jul 2012 19:47
Identification Number:
URI: http://eprints.lancs.ac.uk/id/eprint/51150

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