J. Membrane Biol. 168, 1–8 (1999) The Journal of Membrane Biology © Springer-Verlag New York Inc. 1999 Topical Review K. Kunzelmann, R. Schreiber Physiologisches Institut, Albert-Ludwigs-Universita ¨t Freiburg, Hermann-Herder-Straße 7, 79104 Freiburg, Germany Received: 22 September 1998/Revised: 20 November 1998 Introduction tances are prone to participate in the impaired epithelial electrolyte transport in cystic fibrosis. The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated Cl channel that is defec- + Contribution of CFTR to the Enhanced Na tive in cystic fibrosis. This statement is found in most of Conductance in Cystic Fibrosis the articles somehow dealing with CFTR. However, the initial characterization of this puzzling protein as a con- Enhanced amiloride inhibitable short-circuit currents and ductance regulator is probably more appropriate. As we thus enhanced Na reabsorption has been detected in the have learned over the past few years, CFTR controls the airways of cystic fibrosis patients more than 10 years ago function of various other membrane conductances and [8, 9]. Additional evidence for an enhanced Na con- the list of putative interactions of CFTR with other chan- ductance in airway cells came from patch-clamp studies nels and cellular functions is continuously growing. on primary cultures of CF
The Journal of Membrane Biology – Springer Journals
Published: Mar 1, 1999
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