Research output: Contribution to Journal/Magazine › Journal article › peer-review
Research output: Contribution to Journal/Magazine › Journal article › peer-review
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TY - JOUR
T1 - Macular corneal dystrophy type II
T2 - Multiple studies on a cornea with low levels of sulphated keratan sulphate
AU - Quantock, A J
AU - Fullwood, N J
AU - Thonar, E J M A
AU - Waltman, S R
AU - Capel, M S
AU - Ito, M
AU - Verity, S M
AU - Schanzlin, D J
PY - 1997
Y1 - 1997
N2 - 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.
AB - 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.
KW - cornea
KW - macular corneal dystrophy
KW - proteoglycans
KW - glycosaminoglycans
KW - SYNCHROTRON X-RAY
KW - COLLAGEN FIBRILS
KW - SULFATE PROTEOGLYCAN
KW - ELECTRON-MICROSCOPY
KW - DIFFRACTION
KW - STROMA
KW - GLYCOSAMINOGLYCANS
KW - HETEROGENEITY
KW - THICKNESS
KW - CULTURES
M3 - Journal article
VL - 11
SP - 57
EP - 67
JO - RNIB Eye Contact
JF - RNIB Eye Contact
SN - 0967-8719
ER -