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Sulfation patterns of keratan sulfate in different macular corneal dystrophy immunophenotypes using three different probes
  1. T Saito1,
  2. K Nishida2,
  3. J Nakayama3,
  4. T O Akama4,
  5. M N Fukuda4,
  6. K Watanabe1,
  7. A J Quantock5,
  8. N Maeda1,
  9. H Watanabe1,
  10. Y Tano1
  1. 1
    Department of Ophthalmology, Osaka University Medical School, Osaka, Japan
  2. 2
    Department of Ophthalmology, Tohoku University School of Medicine, Sendai, Japan
  3. 3
    Department of Pathology, Shinshu University School of Medicine, Matsumoto, Japan
  4. 4
    Glycobiology Program, Burnham Institute for Medical Research, La Jolla, CA, USA
  5. 5
    School of Optometry and Vision Sciences, Cardiff University, Cardiff, UK
  1. Professor K Nishida, Department of Ophthalmology, Tohoku University School of Medicine, 1-1 Seiryomachi, Aoba-ku, Sendai, 980-8574, Japan; knishida{at}oph.med.tohoku.ac.jp

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Macular corneal dystrophy (MCD) is subdivided into three immunophenotypes, MCD types I, IA and II, based on the reactivity of serum and corneal tissue to an antibody that recognises sulfated keratan sulfate (KS).1 In MCD type I (MCD-I), antigenic KS is undetectable in both serum and cornea, while in MCD-II it is present at normal or subnormal levels in serum, and is evident immunohistochemically in the corneal stroma. In MCD-IA, antigenic KS is absent from serum and extracellular stromal matrix, but is detected in keratocytes. Mutations of the carbohydrate sulfotransferase gene, CHST6, have been identified as causative for MCD.2 This study investigated the distribution of differentially sulfated KS in cornea in the three main MCD immunophenotypes.

Materials and methods

Serum and postoperative corneal tissue from three unrelated individuals with MCD from …

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