Pflügers Archiv

, Volume 443, Supplement 1, pp S3–S7

Cystic fibrosis and CFTR

  •  R. Greger
  •  R. Schreiber
  •  M. Mall
  •  A. Wissner
  •  A. Hopf
  •  M. Briel
  •  M. Bleich
  •  R. Warth
  •  K. Kunzelmann
Article

DOI: 10.1007/s004240100635

Cite this article as:
Greger, R., Schreiber, R., Mall, M. et al. Pflügers Arch - Eur J Physiol (2001) 443(Suppl 1): S3. doi:10.1007/s004240100635

Abstract.

Cystic fibrosis (CF) is a complex disease affecting epithelial ion transport. There are not many diseases like CF that have triggered such intense research activities. The complexity of the disease is due to mutations in the CFTR protein, now known to be a Cl channel and a regulator of other transport proteins. The various interactions and the large number of disease-causing CFTR mutations is the reason for a variable genotype-phenotype correlation and sometimes unpredictable clinical manifestation. Nevertheless, the research of the past 10 years has resulted in a tremendous increase in knowledge, not only in regard to CFTR but also in regard to molecular interactions and completely new means of ion channel and gene therapy.

Cystic fibrosis CFTR Cystic fibrosis transmembrane conductance regulator ENaC epithelial Na+ channel Epithelium Airways Intestine 

Copyright information

© Springer-Verlag 2001

Authors and Affiliations

  •  R. Greger
    • 1
  •  R. Schreiber
    • 1
  •  M. Mall
    • 1
  •  A. Wissner
    • 1
  •  A. Hopf
    • 1
  •  M. Briel
    • 1
  •  M. Bleich
    • 1
  •  R. Warth
    • 1
  •  K. Kunzelmann
    • 1
  1. 1.Physiologisches Institut, Albert-Ludwigs Universität, Hermann-Herder-Strasse 7, 79104 Freiburg, Germany
  2. 2.School of Biomedical Sciences, Dept. of Physiology and Pharmacology, University of Queensland, QLD 4072, Australia

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