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GLYCOSAMINOGLYCANS OF CARTILAGE AND BONE TISSUE IN TWO CASES OF OSTEOGENESIS IMPERFECTA CONGENITA
Author(s) -
Engfeldt Bengt,
Hjerpe Anders
Publication year - 1976
Publication title -
acta pathologica microbiologica scandinavica section a pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0365-4184
DOI - 10.1111/j.1699-0463.1976.tb00146.x
Subject(s) - glycosaminoglycan , osteogenesis imperfecta , cartilage , hyaluronic acid , chondroitin , chemistry , mineralization (soil science) , bone matrix , chondroitin sulphate , anatomy , biochemistry , medicine , organic chemistry , nitrogen
Epiphyseal cartilage and bone tissue from two cases of osteogenesis imperfecta congenita and one 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 per cent of the chondroitin sulphate disaccharides seemed to be disulphated. The diseased cartilages showed only traces of material with characteristics of disulphated disaccharides. No disulphated disaccharides were isolated from the control materials. No difference between diseased and control material was indicated regarding molecular size, chondroitin‐4‐sulphate/chondroitin‐6‐sulphate ratio or contents of keratan sulphate and hyaluronic acid. The disease is thus 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 organisation of collagen.