European Journal of Pediatrics

, Volume 131, Issue 4, pp 237–245 | Cite as

Link-proteins and non-collagenous proteins from normal and chondrodysplastic cartilages

  • F. Chaminade
  • V. Stanescu
  • R. Stanescu
  • P. Maroteaux
Article
Received:

Abstract

Baboon and human articular and growth cartilage was extracted with 4M guanidinium chloride in the presence of proteolysis inhibitors. After dialysis against 8 M urea pH 6.8 the proteins were separated from proteoglycans by ion-exchange chromatography. The concentrated and reduced protein fractions was analyzed by SDS-PAGE. Bands corresponding to collagen and to 6 major non-collegenous proteins were found. Two of the latter were identified with the link-proteins. By using small columns and microconcentration procedures, a gel-electrophoretic analysis of link-proteins extracted from small pieces of cartilage was performed and ten cases of osteochondrodysplasias were studied. No abnormalities were detected in the following syndromes: achondroplasia, diastrophic dwarfism, thanatophoric dwarfism, Jeune disease, spondyloepiphyseal dysplasia congenita, Kozlowski syndrome, osteogenesis imperfecta, polyepiphyseal dysplasia with diabetes mellitus.

Key words

Cartilage Chondrodysplasia Growth Link-proteins Proteoglycan aggregation Baboon 
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References

  1. 1.
    Anderson, J. C.: Glycoproteins of the connective tissue matrix. Conn. Tiss. Res.7, 251–315 (1976)Google Scholar
  2. 2.
    Antonopoulos, C. A., Axelson, I., Heinegard, D., Gardell, S.: Extraction and purification of proteoglycans from various type of connective tissue. Biochim. Biophys. Acta338, 108–119 (1974)Google Scholar
  3. 3.
    Baker, J., Caterson, B.: The purification and cyanogen bromide cleavage of the link protens from cartilage proteoglycans. Biochem. Biophys. Res. Commun.77, 1–10 (1977)Google Scholar
  4. 4.
    Baker, J., Caterson, B.: The isolation of link proteins from bovine nasal cartilage. Biochim. Biophys. Acta532, 249–258 (1978)Google Scholar
  5. 5.
    Bonnet, F., Perin, J. P., Jolles, P.: Isolation and chemical characterization of two distinct link proteins from bovine nasal cartilage proteoglycan complex. Biochim. Biophys. Acta532, 242–248 (1978)Google Scholar
  6. 6.
    De Luca, S., Heinegard, D., Hascall, V. C.: Chemical and physical changes in proteoglycans during development of chick limb bud grown in vitro. J. Biol. Chem.253, 4713–4722 (1978)Google Scholar
  7. 7.
    Gregory, J. D.: Structure and aggregation of proteoglycans of cartilage in Protides of the biological fluids, Peters, H., ed., Vol. 21, pp. 171–176. Oxford: Pergamon Press 1974Google Scholar
  8. 8.
    Hargingham, T., Muir, H.: The specific interaction of hyaluronic acid with cartilage proteoglycans. Biochim. Biophys. Acta279, 401–405 (1972)Google Scholar
  9. 9.
    Hardingham, T., Muir, H.: Hyaluronic acid in cartilage and proteoglycans aggregation. Biochem. J.139, 565–581 (1974)Google Scholar
  10. 10.
    hascall, V. C., Heinegard, D.: Aggregation of cartilage proteoglycans. I. The role of hyaluric acid. J. Biol. Chem.249, 4232–4243 (1974)Google Scholar
  11. 11.
    Hascall, V. C., Heinegard, D.: Aggregation of cartilage proteoglycans. II. Oligosaccharide competitions of the proteoglycan hyaluronic acid interaction. J. Biol. Chem.249, 4242–4249 (1974)Google Scholar
  12. 12.
    Hascall, V. C., Sajdera, S. W.: Protein-Polysaccharide complex from bovine nasal cartilage. J. Biol. Chem.244, 2384–2396 (1969)Google Scholar
  13. 13.
    Heinergard, D., Hascall, V. C.: Aggregation of cartilage proteoglycans. III. Characteristics of the proteins isolated from trypsin digests of aggregates. J. Biol. Chem.249, 4250–4256 (1974)Google Scholar
  14. 14.
    Keiser, H. D., Shulman, H. J., Sandson, J. I.: Immunochemistry of cartilage proteoglycan. Biochem. J.126, 163–169 (1972)Google Scholar
  15. 15.
    Perin, J. P., Bonnet, F., Jolles, P.: The action of trypsin on purified link proteins from bovine nasal cartilage proteoglycan complex. FEBS letters94, 257–260 (1978)Google Scholar
  16. 16.
    Sajdera, S. W., Hascall, V. C.: Proteinpolysaccaride complex from bovine nasal cartilage. A comparison of low and high ear extraction procedures. J. Biol. Chem.244, 77–87 (1969)Google Scholar
  17. 17.
    Scheidegger, J. J.: Une micro méthode de l'immuno-électrophorèse. Internat. Arch. Allergy Appl. Immunol.7, 103–110 (1955)Google Scholar
  18. 18.
    Stanescu, V., Bona, C., Ionescu, V.: The tibial growing cartilage biopsy in the study of growth disturbances. Acta Endocrin. (Kbh.)64, 577–601 (1970)Google Scholar
  19. 19.
    Stanescu, V., Maroteaux, P.: Gel electrophoretic studies on proteoglycans and collagen of abnormal human cartilage. Pediat. Res.9, 779–782 (1975)Google Scholar
  20. 20.
    Stanescu, V., Maroteaux, P., Sobczak, E.: proteoglycan populations of baboon (Papio Papio) articular cartilage. Biochem. J.163, 103–109 (1977)Google Scholar
  21. 21.
    Stanescu, V., Stanescu, R., Maroteaux, P.: Etude morphologique et biochimique du cartilage de croissance dans les ostéochondrodysplasies. Arch. Franç. Pediat.34, suppl. 3, I-LXXX (1977)Google Scholar
  22. 22.
    Stanescu, V., Stanescu, R., Szirmai, J. A.: Microchemical analysis of the human tibial growth cartilage in various forms of dwarfism. Acta Endocrin. (Kbh.)69, 659–688 (1972)Google Scholar
  23. 23.
    Weber, K., Osborne, M.: The reliability of molecular weight determination by dodecyl sulfate polyacrylamide gel electrophoresis. J. Biol. Chem.244, 4406–4412 (1969)Google Scholar

Copyright information

© Springer-Verlag 1979

Authors and Affiliations

  • F. Chaminade
    • 1
  • V. Stanescu
    • 1
  • R. Stanescu
    • 1
  • P. Maroteaux
    • 1
  1. 1.Unité de Recherches de Génétiques MédicaleHôpital des Enfants-MaladesParisFrance

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