Newly synthesized proteoglycans in pseudoachondroplasia |
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Authors: | M. Beck K. Lingnau J. Spranger |
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Affiliation: | Department of Pediatrics, University of Mainz, Federal Republic of Germany |
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Abstract: | In an attempt to elucidate the biochemical defect in pseudoachondroplasia, proteoglycan metabolism was investigated in cartilage from a patient with the dominant form of this condition. Iliac-crest cartilage was radioactively labeled with 35S-sulfate and the newly synthesized proteoglycans examined for their hydrodynamic size and glycosaminoglycan composition. The banding pattern of the purified proteoglycans was analyzed by gel-electrophoresis using large pore polyacrylamide-agarose. We found a normal chain-length of glycosaminoglycans and a normal ratio of chondroitin-6-sulfate to chondroitin-4-sulfate. The proteoglycans were not enriched in keratan sulfate. Gel electrophoretic analysis of the proteoglycans disclosed a banding pattern comparable to that of two normal controls. In contrast to the findings of other authors no differences between the proteoglycans of pseudoachondroplastic and normal cartilage were detected. |
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Keywords: | Bone Dysplasia Cartilage Glycosaminoglycans Osteochondrodysplasia Proteoglycans Pseudoachondroplasia |
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