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

Processes that modulate the regular architecture and, hence, transpare ncy of the cornea are poorly understood, although proteoglycans are th ought to be involved. Scheie's syndrome displays corneal opacification and systemic accumulation of glycosaminoglycans. The manifestations o f these two occurrences were examined in relation to the corneal strom a. Collagen architecture was investigated by transmission electron mic roscopy and synchrotron x-ray diffraction. Cuprolinic blue staining lo cated sulfated glycosaminoglycan deposits that disrupted the extracell ular matrix. Unlike normal cornea, which contained collagen fibrils of remarkably uniform diameter (26.0 +/- 2.4 nm), there was a large rang e of fibril sizes in the Scheie's syndrome stroma (19.9 to 52.0 nm). M oreover, the distribution of fibril diameters appeared bimodal. X-ray diffraction confirmed the discovery of abnormally large stromal collag en. The results suggest a link in Scheie's syndrome between proteoglyc an content/distribution and stromal disruption, and between stromal di sruption and corneal opacification.