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Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle

Abstract

Dystrophin, the protein encoded by the Duchenne muscular dystrophy (DMD) gene, exists in a large oligomeric complex. We show here that four glycoproteins are integral components of the dystrophin complex and that the concentration of one of these is greatly reduced in DMD patients. Thus, the absence of dystrophin may lead to the loss of a dystrophin-associated glycoprotein, and the reduction in this glycoprotein may be one of the first stages of the molecular pathogenesis of muscular dystrophy.

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References

  1. Hoffman, E. P., Brown, R. H. & Kunkel, L. M. Cell 51, 919–928 (1987).

    Article  CAS  PubMed  Google Scholar 

  2. Zubrzycka-Gaarn, E. E. et al. Nature 333, 466–469 (1988).

    Article  ADS  CAS  PubMed  Google Scholar 

  3. Arahata, K. et al. Nature 333, 861–866 (1988).

    Article  ADS  CAS  PubMed  Google Scholar 

  4. Bonilla, E. et al. Cell 54, 447–452 (1988).

    Article  CAS  PubMed  Google Scholar 

  5. Watkins, S. C., Hoffman, E. P., Slayter, H. S. & Kunkel, L. M. Nature 333, 863–866 (1988).

    Article  ADS  CAS  PubMed  Google Scholar 

  6. Hoffman, E. P. et al. New Engl. J. Med. 318, 1363–1368 (1988).

    Article  CAS  PubMed  Google Scholar 

  7. Cooper, B. J. et al. Nature 334, 154–156 (1988).

    Article  ADS  CAS  PubMed  Google Scholar 

  8. Koenig, M., Monaco, A. P. & Kunkel, L. M. Cell 53, 219–228 (1988).

    Article  CAS  PubMed  Google Scholar 

  9. Davison, M. D. & Critchley, D. R. Cell 52, 159–160 (1988).

    Article  CAS  PubMed  Google Scholar 

  10. Alloisio, N. et al. Biochim. biophys. Acta 816, 57–62 (1985).

    Article  CAS  PubMed  Google Scholar 

  11. Mueller, T. J. & Morrison, M. Erythrocyte Membrane 2: Recent Clinical and Experimental Advances 95–112 (Liss, New York, 1981).

    Google Scholar 

  12. Campbell, K. P. & Kahl, S. D. Nature 338, 259–262 (1989).

    Article  ADS  CAS  PubMed  Google Scholar 

  13. Knudson, C. M., Hoffman, E. P., Kahl, S. D., Kunkel, L. M. & Campbell, K. P. J. biol. Chem. 263, 8480–8484 (1988).

    CAS  PubMed  Google Scholar 

  14. Bonilla, E., Schotland, D. L. & Wakayama, Y. Ann. Neurol. 4, 117–123 (1978).

    Article  CAS  PubMed  Google Scholar 

  15. Capaldi, M. J., Dunn, M. J., Sewry, C. A. & Dubowitz, V. J. neurol. Sci. 63, 129–142 (1984).

    Article  CAS  PubMed  Google Scholar 

  16. Capaldi, M. J., Dunn, M. J., Sewry, C. A. & Dubowitz, V. J. neurol. Sci. 68, 225–231 (1985).

    Article  CAS  PubMed  Google Scholar 

  17. Palek, J. Clin. Haematol. 14, 45–87 (1985).

    CAS  PubMed  Google Scholar 

  18. Alloisio, N., Dorleac, E., Girot, R. & Delaunay, J. hum. Genet. 59, 68–71 (1981).

    Article  CAS  PubMed  Google Scholar 

  19. Tchernia, G., Mohandas, N. & Shohet, S. B. J. clin. Invest. 68, 454–460 (1981).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Turner, P. R., Westwood, T., Regen, C. M. & Steinhardt, R. A. Nature 335, 735–738 (1988).

    Article  ADS  CAS  PubMed  Google Scholar 

  21. Nagy, B. & Samaha, F. J. Ann. Neurol. 20, 50–56 (1986).

    Article  CAS  PubMed  Google Scholar 

  22. Partridge, T. A., Morgan, J. E., Coulton, G. R., Hoffman, E. P. & Kunkel, L. M. Nature 337, 176–179 (1989).

    Article  ADS  CAS  PubMed  Google Scholar 

  23. Francke, U., Darras, B. T., Hersh, J. H., Berg, B. O. & Miller, R. G. Am. J. hum. Genet. 45, 63–72 (1989).

    CAS  PubMed  PubMed Central  Google Scholar 

  24. Zatz, M., Passos-Bueno, M. R. & Rapaport, D. Am. J. med. Genet. 32, 407–410 (1989).

    Article  CAS  PubMed  Google Scholar 

  25. Mitchell, R. D., Palade, P. & Fleischer, S. J. Cell Biol. 95, 1008–1016 (1983).

    Article  Google Scholar 

  26. Imagawa, T., Smith, J. S., Coronado, R. & Campbell, K. P. J. biol. Chem. 262, 16636–16643 (1987).

    CAS  PubMed  Google Scholar 

  27. Laemmli, U. K. Nature 227, 680–685 (1970).

    Article  ADS  CAS  PubMed  Google Scholar 

  28. Strynadka, N. C. J., Redmond, M. J., Parker, J. M. R., Scraba, D. G. & Hodges, R. S. J. Virol. 62, 3474–3483 (1988).

    CAS  PubMed  PubMed Central  Google Scholar 

  29. Jorgensen, A. O. et al. Cell Motil. Cytoskel. 9, 164–174 (1988).

    Article  CAS  Google Scholar 

  30. Campbell, K. P. et al. J. biol. Chem. 262, 6460–6463 (1987).

    CAS  PubMed  Google Scholar 

  31. Casadei, J. M. & Barchi, R. L. J. Neurochem. 48, 773–778 (1987).

    Article  CAS  PubMed  Google Scholar 

  32. Sharp, A. H. & Campbell, K. P. J. biol. Chem. 264, 2816–2825 (1989).

    CAS  PubMed  Google Scholar 

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Ervasti, J., Ohlendieck, K., Kahl, S. et al. Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle. Nature 345, 315–319 (1990). https://doi.org/10.1038/345315a0

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