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Science 29 March 1996:
Vol. 271. no. 5257, pp. 1876 - 1879
DOI: 10.1126/science.271.5257.1876
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Reports

Failure of the Cystic Fibrosis Transmembrane Conductance Regulator to Conduct ATP

M. M. Reddy, P. M. Quinton, C. Haws, J. J. Wine, R. Grygorczyk, J. A. Tabcharani, J. W. Hanrahan, K. L. Gunderson, R. R. Kopito *

The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride ion channel regulated by protein kinase A and adenosine triphosphate (ATP). Loss of CFTR-mediated chloride ion conductance from the apical plasma membrane of epithelial cells is a primary physiological lesion in cystic fibrosis. CFTR has also been suggested to function as an ATP channel, although the size of the ATP anion is much larger than the estimated size of the CFTR pore. ATP was not conducted through CFTR in intact organs, polarized human lung cell lines, stably transfected mammalian cell lines, or planar lipid bilayers reconstituted with CFTR protein. These findings suggest that ATP permeation through the CFTR is unlikely to contribute to the normal function of CFTR or to the pathogenesis of cystic fibrosis.

M. M. Reddy and P. M. Quinton, Division of Biomedical Sciences, University of California, Riverside, CA 92521, USA.
C. Haws and J. J. Wine, Cystic Fibrosis Research Laboratory, Department of Psychology, Stanford University, Stanford, CA 94305, USA.
R. Grygorczyk, J. A. Tabcharani, J. W. Hanrahan, Department of Physiology, McGill University, Montreal, Quebec, H3G 1Y6, Canada.
K. L. Gunderson and R. R. Kopito, Department of Biological Sciences, Stanford University, Stanford, CA 94305-5020, USA.
* To whom correspondence should be addressed.


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