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The distribution of sulphated proteoglycans within the stromas of three patients (A,B,C) suffering from macular corneal dystrophy was studied using the specific dye Cuprolinic Blue in a 'critical electrolyte concentration' method. The corneas were examined using transmission electron microscopy and A and C were further studied by low-angle synchroton X-ray diffraction. Sera from all three patients were analyzed for the presence of keratan sulphate using a monoclonal antibody in an enzyme-linked immunosorbent assay. The serum from Patient A contained keratan sulphate, but the chains were thought to be shorter or less sulphate in their sera. Electron microscopy showed many electron-transparent lacunae randomly distributed throughout the specimens. The average collagen fibril diameter was normal but there were differences in packing between the specimens. Specimen A was closely-packed with most collagen fibrils in contact with their neighbours. Specimens B and C showed fewer regions of close packing; in most of the tissue the interfibrillar spacing appeared normal. Staining with Cuprolinic Blue revealed an unusual distribution of proteoglycans in some parts of the interfibrillar matrix, particularly in A, with 'small' proteoglycans running exclusively parallel to the collagen fibrils. Furthermore in A, and to a lesser extent in B and C, some lacunae were filled with clusters of abnormal sulphated proteoglycan filaments (of various sizes) which were chondroitinase ABC susceptible. Clearly defined regions, both within the lacunae and elsewhere, failed to stain with Cuprolinic Blue; this suggests an absence of sulphated proteoglycans within these areas. Equatorial X-ray diffraction of the wet tissues (A and C) gave values for the mean interfibrillar centre-to-centre separation of 43 +/- 2 nm in Specimen A and 52 +/- 3 nm in Specimen C. The differences observed in the serum keratan sulphate levels, the packing of the collagen fibrils and the distribution of chondroitin/dermatan sulphate proteoglycans confirm the heterogeneity that exists within the macular corneal dystrophies.
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
October 1982,
Journal of molecular biology,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
April 1992,
Investigative ophthalmology & visual science,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
February 1978,
Journal of molecular biology,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
January 2004,
Annual review of biophysics and biomolecular structure,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
July 1987,
Current eye research,
K M Meek, and
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A E Ridgway, and
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E J Thonar
January 1997,
Advances in protein chemistry,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
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E J Thonar
January 1986,
Connective tissue research,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
July 1979,
The Journal of cell biology,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
February 1957,
Biochimica et biophysica acta,
K M Meek, and
A J Quantock, and
G F Elliott, and
A E Ridgway, and
A B Tullo, and
A J Bron, and
E J Thonar
February 2012,
Reports on progress in physics. Physical Society (Great Britain),
