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Science 3 February 1989:
Vol. 243. no. 4891, pp. 657 - 660
DOI: 10.1126/science.2464852
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Articles

Science, Vol 243, Issue 4891, 657-660
Copyright © 1989 by American Association for the Advancement of Science

articles

A cAMP-regulated chloride channel in lymphocytes that is affected in cystic fibrosis

JH Chen, H Schulman, and P Gardner

Department of Medicine, Stanford University, CA 94305.

A defect in regulation of a chloride channel appears to be the molecular basis for cystic fibrosis (CF), a common lethal genetic disease. It is shown here that a chloride channel with kinetic and regulatory properties similar to those described for secretory epithelial cells is present in both T and B lymphocyte cell lines. The regulation of the channels by adenosine 3',5'-monophosphate (cAMP)-dependent protein kinase in transformed B cells from CF patients is defective. Thus, lymphocytes may be an accessible source of CF tissue for study of this defect, for cloning of the chloride channel complex, and for diagnosis of the disease.


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Lung Infections Associated with Cystic Fibrosis.
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Electrolyte Transport in the Mammalian Colon: Mechanisms and Implications for Disease.
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A src-like kinase activates outwardly rectifying chloride channels in CFTR-defective lymphocytes.
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Tyrosine kinase-dependent activation of a chloride channel in CD95-induced apoptosis in T lymphocytes.
I. Szabo, A. Lepple-Wienhues, K. N. Kaba, M. Zoratti, E. Gulbins, and F. Lang (1998)
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Outwardly rectifying Cl- channel in guinea pig small intestinal villus enterocytes: effect of inhibitors.
A. S. Monaghan, G. M. Mintenig, and F. V. Sepulveda (1997)
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CD39 Is an Ecto-(Ca[IMAGE],Mg[IMAGE])-apyrase.
T.-F. Wang and G. Guidotti (1996)
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Cell cycle dependence of chloride permeability in normal and cystic fibrosis lymphocytes.
J. Bubien, K. Kirk, T. Rado, and R. Frizzell (1990)
Science 248, 1416-1419
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Purification and reconstitution of chloride channels from kidney and trachea.
D. Landry, M. Akabas, C Redhead, A Edelman, E. Cragoe Jr, and Q Al-Awqati (1989)
Science 244, 1469-1472
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