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Sulphation patterns of keratan sulphate proteoglycan in sclerocornea resemble cornea rather than sclera
  1. R D Young1,
  2. A J Quantock1,
  3. C Sotozono2,
  4. N Koizumi2,3,
  5. S Kinoshita2
  1. 1Structural Biophysics Group, School of Optometry and Vision Sciences, Cardiff University, Cardiff, UK
  2. 2Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan
  3. 3Research Center for Regenerative Medicine, Doshisha University, Kyoto, Japan
  1. Correspondence to: Professor Shigeru Kinoshita Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kawaramachi-hirokoji, kamigyo-ku, Kyoto 602-0841, Japan; shigeruk{at}ophth.kpu-m.ac.jp

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Sclerocornea is one of the most frequent causes of congenital blindness,1 in which the cornea is opaque and resembles sclera at birth.2 The stromal matrix in sclera and in cornea is composed of collagen fibrils, with proteoglycans (PGs) in the interfibrillar space fulfilling important roles in relation to tissue structure and function. Sclera and cornea contain distinct PG populations. Whereas the predominant corneal PG carries highly sulphated keratan sulphate (KS) glycosaminoglycans (GAGs), sulphated KSPGs are not major scleral components.3 Here, we investigate if sclerocornea is more like sclera or cornea in terms of its KSPG molecular profile.

CASE REPORT

An 8 month old girl, born at 39 weeks after an uncomplicated pregnancy and delivery, had bilateral corneal opacification and was referred to Kyoto Prefectural University of Medicine. There was no family history of eye disease. Slit lamp examination disclosed diffuse, full thickness opacification, which was more severe in her left eye (fig 1A), preventing examination of the anterior chamber. Ultrasound biomicroscopy detected synechiae between …

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