Macular corneal dystrophy type II

Multiple studies on a cornea with low levels of sulphated keratan sulphate

Andrew J. Quantock, Nigel J. Fullwood, Eugene J M A Thonar, Stephen R. Waltman, Malcolm S. Capel, Mitsutoshi Ito, Steven Verity, David J. Schanzlin

Research output: Contribution to journalArticle

17 Citations (Scopus)

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 Å 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 nm 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.

Original languageEnglish (US)
Pages (from-to)57-67
Number of pages11
JournalEye
Volume11
Issue number1
StatePublished - 1997

Fingerprint

Keratan Sulfate
Cornea
Collagen
keratan-sulfate endo-1,4-beta-galactosidase
Proteoglycans
Chondroitin ABC Lyase
Peptide-N4-(N-acetyl-beta-glucosaminyl) Asparagine Amidase
Descemet Membrane
Vacuoles
Sulfur
Endothelium
Extracellular Matrix
Digestion
X-Rays
Type II Macular Corneal Dystrophy

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems

Cite this

Quantock, A. J., Fullwood, N. J., Thonar, E. J. M. A., Waltman, S. R., Capel, M. S., Ito, M., ... Schanzlin, D. J. (1997). Macular corneal dystrophy type II: Multiple studies on a cornea with low levels of sulphated keratan sulphate. Eye, 11(1), 57-67.

Macular corneal dystrophy type II : Multiple studies on a cornea with low levels of sulphated keratan sulphate. / Quantock, Andrew J.; Fullwood, Nigel J.; Thonar, Eugene J M A; Waltman, Stephen R.; Capel, Malcolm S.; Ito, Mitsutoshi; Verity, Steven; Schanzlin, David J.

In: Eye, Vol. 11, No. 1, 1997, p. 57-67.

Research output: Contribution to journalArticle

Quantock, AJ, Fullwood, NJ, Thonar, EJMA, Waltman, SR, Capel, MS, Ito, M, Verity, S & Schanzlin, DJ 1997, 'Macular corneal dystrophy type II: Multiple studies on a cornea with low levels of sulphated keratan sulphate', Eye, vol. 11, no. 1, pp. 57-67.
Quantock AJ, Fullwood NJ, Thonar EJMA, Waltman SR, Capel MS, Ito M et al. Macular corneal dystrophy type II: Multiple studies on a cornea with low levels of sulphated keratan sulphate. Eye. 1997;11(1):57-67.
Quantock, Andrew J. ; Fullwood, Nigel J. ; Thonar, Eugene J M A ; Waltman, Stephen R. ; Capel, Malcolm S. ; Ito, Mitsutoshi ; Verity, Steven ; Schanzlin, David J. / Macular corneal dystrophy type II : Multiple studies on a cornea with low levels of sulphated keratan sulphate. In: Eye. 1997 ; Vol. 11, No. 1. pp. 57-67.
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