Abstract
Summary: Biochemical analysis and morphologic observation of the iliac crest cartilage from 8-year-old girl with I-cell disease were performed. Instead of hypertrophic change of chondrocytes in the zone of cartilage-bone junction, there were small atrophic cells having many inclusion bodies accumulated with lamellar materials. Another characteristic finding in this zone was munerous cellular debris in the cartiage matrix. Significantly, a large amount of unsulfated chondroitin (21%) occurred in the cartilage with I-cell tisease, and compensatory decrease of chondroitin 4-sulfate (14%) was observed. The percentage of chondroitin and chondroitin
Speculation: Occurrence of unsulfated chondroitin in cartilage cartilage matrix may reflect abnormality in the process of posttranslational assembly and modification of glycosaminoglycans in I-cell disease. Increase of cellular debris as well as appearance of atrophic chondrocytes in the zone of cartilage-bone junction may have relation with defective growth of the skeleton.
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Nogami, H., Oohira, A., Suzuki, F. et al. Cartilage of I-Cell Disease. Pediatr Res 15, 330–334 (1981). https://doi.org/10.1203/00006450-198104000-00008
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DOI: https://doi.org/10.1203/00006450-198104000-00008
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