GLYCOSAMINOGLYCANS OF CARTILAGE AND BONE TISSUE IN TWO CASES OF OSTEOGENESIS IMPERFECTA CONGENITA

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Abstract
Epiphyseal cartilage and bone tissue from 2 cases of osteogenesis imperfecta congenita and 1 control case with similar skeletal age were examined regarding their glycosaminoglycan content. The diseased bone tissue showed a 3-fold increase in glycosaminoglycans, and 20-25% of the chondroitin sulfate disaccharides were disulfated. The diseased cartilages showed only traces of material with characteristics of disulfated disaccharides. No disulfated disaccharides were isolated from the control materials. No difference between diseased and control material was indicated regarding molecular size, chondroitin-4-sulfate/chondroitin-6-sulfate ratio or contents of keratan sulfate and hyaluronic acid. The disease is associated with fundamental changes of the glycosaminoglycan structure, and these changes may be of functional importance to the mineralization process as well as to the organization of collagen.