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CFTR Folding and Maturation in Cells

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Part of the book series: Methods in Molecular Medicine™ ((MIMM,volume 70))

Abstract

Structural predictions suggest that cystic fibrosis transmembrane conductance

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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, 1066–1073.

    Article  CAS  PubMed  Google Scholar 

  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, 827–834.

    Article  CAS  PubMed  Google Scholar 

  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, 6076–6086.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  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, 761–764.

    Article  Google Scholar 

  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, 180–183.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  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, 129–135.

    Article  CAS  PubMed  Google Scholar 

  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, 137–142.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  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, 321–327.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  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, 2433–2444.

    CAS  PubMed  Google Scholar 

  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, 831–837.

    CAS  PubMed  Google Scholar 

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

    Google Scholar 

  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. 240 413–425.

    Article  CAS  PubMed  Google Scholar 

  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, 21,873–21,877.

    Article  CAS  PubMed  Google Scholar 

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© 2002 Humana Press Inc.

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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 Medicine™, vol 70. Humana Press. https://doi.org/10.1385/1-59259-187-6:229

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  • 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

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