Skip to main content

CFTR Folding and Maturation in Cells

  • Protocol
Cystic Fibrosis Methods and Protocols

Part of the book series: Methods in Molecular Medicineā„¢ ((MIMM,volume 70))

Abstract

Structural predictions suggest that cystic fibrosis transmembrane conductance

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

Access this chapter

Protocol
USD 49.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Riordan, J. R., Rommens, J. M., Kerem, B., Alon, M., Rosmahel, R., Grzelchak, Z., et al. (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245, 1066ā€“1073.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  2. Cheng, S. H., Gregory, R. J., Marshall, J., Paul, S., Souza, D. W., White, G. A., et al. (1990) Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63, 827ā€“834.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  3. Lukacs, G. L., Mohamed, A., Kartner, N., Chang, X.-B., Riordan, J. R., and Grinstein, S. (1994) Conformational maturation of CFTR but not its mutant counterpart (DF508) occurs in the endoplasmic reticulum and requires ATP. EMBO J. 13, 6076ā€“6086.

    CASĀ  PubMedĀ  Google ScholarĀ 

  4. Ward, C. L. and Kopito, R. R. (1994) Intracellular turnover of cystic fibrosis transmembrane conductance regulator. J Biol. Chem. 269, 25,710ā€“25,718.243

    CASĀ  PubMedĀ  Google ScholarĀ 

  5. Zielenski, J. and Tsui, L.-C. (1995) Cystic fibrosis: genotypic and phenotypic variations. Annu. Rev. Genet. 29, 777ā€“807.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  6. Denning, G. M., Anderson, M. P., Amara, J. F., Marshall, J., Smith, A. E., and Welsh, M. J. (1992) Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature sensitive. Nature 350, 761ā€“764.

    ArticleĀ  Google ScholarĀ 

  7. Zhang, F., Kartner, N., and Lukacs, G. L. (1998) Limited proteolysis as a probe for arrested conformational maturation of AF508 CFTR. Nature Struct. Biol. 5, 180ā€“183.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  8. Ward, C. L., Omura, S., and Kopito, R. R. (1995) Degradation of CFTR by the ubiqutin-proteasome pathway. Cell 83, 121ā€“128.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  9. Jensen, T. J., Loo, M. A., Pind, S., Williams, D. B., Goldberg, A. L., and Riordan, J. R. (1995) Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 83, 129ā€“135.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  10. Chang, X. B., Cui, L., Hou, Y. X., Jensen, T. J., Aleksandov, A. A., Mengos, A., and Riordan, J. R. (1999) Removal of multiple arginine-framed trafficking signals overcomes misprocessing of AF508 CFTR present in most patients with cystic fibrosis. Mol. Cell. 4, 137ā€“142.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  11. Hammond, C. and Helenius, A. (1995) Quality control in the secretory pathway. Curr. Opin. Cell Biol. 7, 523ā€“529.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  12. Kartner, N., Augustinas, O., Jensen, T. J., Naismith, A. L., and Riordan, J. R. (1992) Mislocalization of DF508 CFTR in cystic fibrosis sweat gland. Nature Genet. 1, 321ā€“327.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  13. Kartner, N. and Riordan, J. R. (1998) Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator. Meth. Enzym. 292, 629ā€“653.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  14. Walker, J., Watson, J., Holmes, C., Edelman, A., and Banting, G. (1995) Production and characterization of monoclonal and polyclonal antibodies to different regions of the CFTR: detection of immunologically related proteins. J Cell Sci. 108, 2433ā€“2444.

    CASĀ  PubMedĀ  Google ScholarĀ 

  15. Claass, A., Sommer, M., de Jonge, H., Kalin, N., and Tummler, B. (2000) Applicability of diferent antibodies for immunohistochemical localization of CFTR in sweat glands from healthy controls and from patients with cystic fibrosis..J. Histochem. Cytochem. 48, 831ā€“837.

    CASĀ  PubMedĀ  Google ScholarĀ 

  16. Beynon, R. J. and Bond, J. S. (eds.) (1989) Proteolytic Enzymes, A Practical Approach, IRL Press, Oxford.

    Google ScholarĀ 

  17. Balch, W. E. and Rothman, J. E. (1985) Characterization of protein transport between successive compartments of the Golgi apparatus: asymetric properties of donor and acceptor activities in a cell free system. Arch. Biochem. Biophys. 240 413ā€“425.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

  18. Haardt, M., Benharouga, M., Lechardeur, D., Kartner, N., and Lukacs, G. L. (1999) C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. J. Biol. Chem. 274, 21,873ā€“21,877.

    ArticleĀ  CASĀ  PubMedĀ  Google ScholarĀ 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

Ā© 2002 Humana Press Inc.

About this protocol

Cite this protocol

Benharouga, M., Sharma, M., Lukacs, G.L. (2002). CFTR Folding and Maturation in Cells. In: Skach, W.R. (eds) Cystic Fibrosis Methods and Protocols. Methods in Molecular Medicineā„¢, vol 70. Humana Press. https://doi.org/10.1385/1-59259-187-6:229

Download citation

  • DOI: https://doi.org/10.1385/1-59259-187-6:229

  • Publisher Name: Humana Press

  • Print ISBN: 978-0-89603-897-4

  • Online ISBN: 978-1-59259-187-9

  • eBook Packages: Springer Protocols

Publish with us

Policies and ethics