Skip to main content
SpringerLink
Log in

Arrest of CFTRΔF508 folding

  • News & Views
  • Published:

From Nature Structural & Molecular Biology

View current issue Submit your manuscript

The deletion of residue 508 in CFTR is the most common cystic fibrosis–causing mutation. Recent studies indicate that the main chain and side chain of this residue contribute to the proper folding of CFTR at different stages.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Figure 1: Domain structure of CFTR.
Figure 2: Misfolding and selection of CFTRΔF508 for degradation.

References

  1. Dobson, C.M. Nature 426, 884–890 (2003).

    Article  CAS  Google Scholar 

  2. Cyr, D.M., Hohfeld, J. & Patterson, C. Trends Biochem. Sci. 27, 368–375 (2002).

    Article  CAS  Google Scholar 

  3. Welsh, M.J. & Ostedgaard, L.S. Nat. Struct. Biol. 5, 167–169 (1998).

    Article  CAS  Google Scholar 

  4. Meacham, G.C. et al. EMBO J. 18, 1492–1505 (1999).

    Article  CAS  Google Scholar 

  5. Gelman, M.S. & Kopito, R.R. J. Clin. Invest. 110, 1591–1597 (2002).

    Article  CAS  Google Scholar 

  6. Du, K., Sharma, M. & Lukacs, G.L. Nat. Struct. Mol. Biol. 12, 17–25 (2005).

    Article  CAS  Google Scholar 

  7. Thibodeau, P., Brautigam, C.A., Machius, M. & Thomas, P.J. 12, 10–16 (2005).

  8. Riordan, J.R. et al. Science 245, 1066–1073 (1989).

    Article  CAS  Google Scholar 

  9. Lewis, H.A. et al. EMBO J. 23, 282–293 (2004).

    Article  CAS  Google Scholar 

  10. Smith, P.C. et al. Mol. Cell 10, 139–249 (2002).

    Article  CAS  Google Scholar 

  11. Lewis, H.A. et al. J. Biol. Chem. published online, 3 November 2004 (doi:10.1074/jbc.M410968200).

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

    Article  CAS  Google Scholar 

  13. Chang, G. & Roth, C.B. Science 293, 1793–1800 (2001).

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  15. Tector, M. & Hartl, F.U. EMBO J. 18, 6290–6298 (1999).

    Article  CAS  Google Scholar 

  16. Chen, E.Y., Bartlett, M.C., Loo, T.W. & Clarke, D.M. J. Biol. Chem. 279, 39620–39627 (2004).

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  18. Meacham, G.C., Patterson, C., Zhang, W., Younger, J.M. & Cyr, D.M. Nat. Cell Biol. 3, 100–105 (2001).

    Article  CAS  Google Scholar 

  19. Younger, J.M. et al. J. Cell Biol. 167, 1075–1085 (2004).

Download references

Acknowledgements

Research in the laboratory of D.M.C. is supported by the US National Institutes of Health and Cystic Fibrosis Foundation.

Author information

Authors and Affiliations

  1. Department of Cell and Developmental Biology and the North Carolina Cystic Fibrosis Research Center, The University of North Carolina, Chapel Hill, 27599, North Carolina, USA

    Douglas M Cyr

Authors
  1. Douglas M Cyr
    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

Cyr, D. Arrest of CFTRΔF508 folding. Nat Struct Mol Biol 12, 2–3 (2005). https://doi.org/10.1038/nsmb0105-2

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1038/nsmb0105-2

  • Springer Nature America, Inc.

This article is cited by

Search

Navigation