Absence of normal keratan sulfate in the blood of patients with macular corneal dystrophy

Eugene J.M.A. Thonar, Roger F. Meyer, Richard F. Dennis, Mary Ellen Lenz, Brian Maldonado, John R. Hassell, A. Tyl Hewitt, Walter J. Stark, E. Lee Stock, Klaus E. Kuettner, Gordon K. Klintworth

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54 Scopus citations


We measured levels of sulfated keratan sulfate in serum using a monoclonal antibody in an enzyme-linked immunosorbent assay. Sulfated keratan sulfate was not detected in the serum of 16 patients with macular corneal dystrophy, but was present at normal levels in 66 patients with other corneal diseases. There were no differences with respect to age, sex, and other ocular findings. This monoclonal antibody recognizes a sulfated carbohydrate epitope present in both corneal and skeletal keratan sulfate. Since most serum keratan sulfate is derived from the cartilages, the defect in keratan sulfate synthesis in macular corneal dystrophy may not be restricted to corneal cells. This assay should prove useful in the diagnosis of macular corneal dystrophy, particularly in children at risk before the appearance of opacification.

Original languageEnglish (US)
Pages (from-to)561-569
Number of pages9
JournalAmerican journal of ophthalmology
Issue number5
StatePublished - Nov 15 1986
Externally publishedYes

ASJC Scopus subject areas

  • Ophthalmology


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