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.

  • News & Views
  • Published:

Cystic fibrosis problem probed by proteolysis

Nature Structural Biology volume 5, pages 167–169 (1998)Cite this article

The cystic fibrosis mutation, ΔF508, disrupts CFTR biosynthesis. ΔF508 protein resembles an early intermediate in wild type folding, but fails to adopt a mature conformation and hence cannot escape the ER.

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

References

  1. Welsh, M.J. & Smith, A.E. Cell 73, 1251–1254 (1993).

    Article  CAS  Google Scholar 

  2. Zhang, F., Kartner, N. & Lukacs, G.L. Nature Struct. Biol. 5, 180–183 (1998).

    Article  CAS  Google Scholar 

  3. Welsh, M.J., Tsui, L.-C., Boat, T.F. & Beaudet, A.L. In The metabolic and molecular basis of inherited disease (eds Scriver, C.R., Beaudet, A.L., Sly, W.S. & Valle, D.) 3799–3876 (McGraw-Hill, Inc., New York; 1995).

    Google Scholar 

  4. Cheng, S.H. et al. Cell 63, 827–834 (1990).

    Article  CAS  Google Scholar 

  5. Lukacs, G.L. et al. EMBO J. 13, 6076–6086 (1994).

    Article  CAS  Google Scholar 

  6. Ward, C.L. & Kopito, R.R. J. Biol. Chem. 269, 25710–25718 (1994).

    CAS  PubMed  Google Scholar 

  7. Ward, C.L., Omura, S. & Kopito, R.R. Cell 83, 121–127 (1995).

    Article  CAS  Google Scholar 

  8. Jensen, T.J. et al. Cell 83, 129–135 (1995).

    Article  CAS  Google Scholar 

  9. Qu, B-H. & Thomas, P.J. J. Biol. Chem. 271, 7261–7264 (1996).

    Article  CAS  Google Scholar 

  10. Qu, B.-H., Strickland, E.H. & Thomas, P.J. J. Biol. Chem. 272, 15739–15744 (1997).

    Article  CAS  Google Scholar 

  11. Yang, Y., Janich, S., Cohn, J.A. & Wilson, J.M. Proc. Natl. Acad. Sci. USA 90, 9480–9484 (1993).

    Article  CAS  Google Scholar 

  12. Pasyk, E.A. & Foskett, J.K. J. Biol. Chem. 270, 12347–12350 (1995).

    Article  CAS  Google Scholar 

  13. Denning, G.M. et al. Nature 358, 761–764 (1992).

    Article  CAS  Google Scholar 

  14. Brown, C.R., Hong-Brown, L.Q., Biwersi, J., Verkman, A.S. & Welch, W.J. Cell Stress Chaperon. 1, 117–125 (1996).

    Article  CAS  Google Scholar 

  15. Teem, J.L. et al. Cell 73, 335–346 (1993).

    Article  CAS  Google Scholar 

  16. Chu, C-S., Trapnell, B.C., Curristin, S., Cutting, G.R. & Crystal, R.G. Nature Genet. 3, 151–156 (1993).

    Article  CAS  Google Scholar 

  17. Veeze, H.J. et al. J. Clin. Invest. 93, 461–466 (1994).

    Article  CAS  Google Scholar 

  18. Sheppard, D.N., Ostedgard L.S., Winter, M.C., and Welsh, M. J. EMBOJ 14, 876–883 (1995).

    Article  CAS  Google Scholar 

  19. Lansbury, P.T. Jr., Neuron 19, 1151–1154 (1997).

    Article  CAS  Google Scholar 

  20. Ross, C.A. Neuron 19, 1147–1150 (1997).

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. the Howard Hughes Medical Institute, Departments of Internal Medicine and Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa, 52245, USA

    Michael J. Welsh & Lynda S. Ostedgaard

Authors
  1. Michael J. Welsh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lynda S. Ostedgaard
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michael J. Welsh.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Welsh, M., Ostedgaard, L. Cystic fibrosis problem probed by proteolysis. Nat Struct Mol Biol 5, 167–169 (1998). https://doi.org/10.1038/nsb0398-167

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1038/nsb0398-167

This article is cited by

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