Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Letter
  • Published:

Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse

Nature volume 349, pages 69–71 (1991)Cite this article

Abstract

HUMAN X-linked Duchenne and Becker muscular dystrophies are due to defects in dystrophin, the product of an exceptionally large gene1,2. Although dystrophin has been characterized as a spectrin-like3 submembranous4 cytoskeletal protein, there is no experimental evidence for its function in the structural maintenance of muscle5. Current hypotheses attribute necrosis of dystrophin-less fibres in situ to mechanical weakening of the outer membrane6, to an excessive influx of Ca2+ions7,8, or to a combination of these two mechanisms, possibly mediated by stretch-sensitive ion channels9. Using hypo-osmotic shock to determine stress resistance10 and a mouse model (mdx)11,12 for the human disease, we show that functional dystrophin contributes to the stability of both cultured myotubes and isolated mature muscle fibres.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Koenig, M. et al. Cell 50, 509–517 (1987).

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  3. Koenig, M. & Kunkel, L. M. J. biol. Chem. 265, 4560–4566 (1990).

    CAS  PubMed  Google Scholar 

  4. Cullen, M. J., Walsh, J., Nicholson, L. V. B. & Harris, J. B. Proc. R. Soc. B240, 197–210 (1990).

    Article  ADS  CAS  Google Scholar 

  5. Slater, C. R. Curr. Opinion Cell Biol. 1, 110–114 (1989).

    Article  CAS  PubMed  Google Scholar 

  6. Karpati, G. & Carpenter, S. Am. J. Med. Genet. 25, 653–658 (1986).

    Article  CAS  PubMed  Google Scholar 

  7. Duncan, C. J. Experientia 45, 175–177 (1989).

    Article  CAS  PubMed  Google Scholar 

  8. Turner, P. R., Westwood, T., Regen, C. M. & Steinhardt, R. A. Nature 335, 735–738 (1989).

    Article  ADS  Google Scholar 

  9. Franco, A. & Lansman, J. B. Nature 344, 670–673 (1990).

    Article  ADS  CAS  PubMed  Google Scholar 

  10. Menke, A. & Jockusch, H. J. neurol. Sci. (sup.) 98, 124 (1990).

    Google Scholar 

  11. Bulfield, G., Siller, W. G., Wight, P. A. L. & Moore, K. J. Proc. natn. Acad. Sci. U.S.A. 81, 1189–1192 (1984).

    Article  ADS  CAS  Google Scholar 

  12. Sicinski, P. et al. Science 244, 1578–1580 (1989).

    Article  ADS  CAS  PubMed  Google Scholar 

  13. Koeltgen, D., Brinkmeier, H. & Jockusch, H. Muscle & Nerve (in the press).

  14. Watkins, S. C., Hoffman, E. P., Slayter, H. S. & Kunkel, L. M. Muscle & Nerve 12, 861–868 (1989).

    Article  CAS  Google Scholar 

  15. Jockusch, H., Friedrich, G. & Zippel, M. Muscle & Nerve 13, 551–555 (1990).

    Article  CAS  Google Scholar 

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

    Article  ADS  CAS  PubMed  Google Scholar 

  17. Jockusch, H. in The Dynamic State of Muscle Fibers (ed. Pette, D.) 429–443 (de Gruyter, New York, 1990).

    Google Scholar 

  18. Carnwath, J. W. & Shotton, D. M. J. neurol. Sci. 80, 39–54 (1987).

    Article  CAS  PubMed  Google Scholar 

  19. Foster, A. H. & Carlson, B. M. Anesthetics 58, 727–736 (1980).

    Google Scholar 

  20. Cooper, B. J. & Hamill, O. P. Soc. Neurosci. (Abstr.) 15, 412.5 (1989).

    Google Scholar 

  21. Ervasti, J. M., Ohlendieck, K., Kahl, S. D., Gaver, M. G. & Campbell, K. P. Nature 345, 315–319 (1990).

    Article  ADS  CAS  PubMed  Google Scholar 

  22. Blau, H. M. & Webster, C. Proc. natn. Acad. Sci. U.S.A. 71, 5623–5627 (1981).

    Article  ADS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Developmental Biology Unit, University of Bielefeld, POB 8640, W-4800, Bielefeld, 1, Germany

    A. Menke & H. Jockusch

Authors
  1. A. Menke
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. Jockusch
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Menke, A., Jockusch, H. Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature 349, 69–71 (1991). https://doi.org/10.1038/349069a0

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/349069a0

This article is cited by

Comments

By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing