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SCHEIES-SYNDROME:THE ARCHITECTURE OF CORNEAL COLLAGEN AND DISTRIBUTION OF CORNEAL PROTEOGLYCANS

QUANTOCK, A J and MEEK, K M and FULLWOOD, N J and ZABEL, R W (1993) SCHEIES-SYNDROME:THE ARCHITECTURE OF CORNEAL COLLAGEN AND DISTRIBUTION OF CORNEAL PROTEOGLYCANS. Canadian Journal of Ophthalmology, 28 (6). pp. 266-272. ISSN 0008-4182

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Abstract

Processes that modulate the regular architecture and, hence, transparency of the cornea are poorly understood, although proteoglycans are thought to be involved. Scheie's syndrome displays corneal opacification and systemic accumulation of glycosaminoglycans. The manifestations of these two occurrences were examined in relation to the corneal stroma. Collagen architecture was investigated by transmission electron microscopy and synchrotron x-ray diffraction. Cuprolinic blue staining located sulfated glycosaminoglycan deposits that disrupted the extracellular matrix. Unlike normal cornea, which contained collagen fibrils of remarkably uniform diameter (26.0 +/- 2.4 nm), there was a large range of fibril sizes in the Scheie's syndrome stroma (19.9 to 52.0 nm). Moreover, the distribution of fibril diameters appeared bimodal. X-ray diffraction confirmed the discovery of abnormally large stromal collagen. The results suggest a link in Scheie's syndrome between proteoglycan content/distribution and stromal disruption, and between stromal disruption and corneal opacification.

Item Type: Article
Journal or Publication Title: Canadian Journal of Ophthalmology
Uncontrolled Keywords: CORNEA ; STROMA ; PROTEOGLYCANS ; SCHEIE SYNDROME ; SYNCHROTRON ; X-RAY-DIFFRACTION ; ELECTRON-MICROSCOPY ; V COLLAGEN ; DYSTROPHY ; SULFATE ; INVITRO ; FIBRILS ; FIBRILLOGENESIS ; ORGANIZATION
Subjects: UNSPECIFIED
Departments: Faculty of Health and Medicine > Biomedical & Life Sciences
ID Code: 49173
Deposited By: ep_importer_pure
Deposited On: 22 Aug 2011 16:44
Refereed?: Yes
Published?: Published
Last Modified: 09 Apr 2014 22:38
Identification Number:
URI: http://eprints.lancs.ac.uk/id/eprint/49173

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