Volume 272, Number 12, Issue of March 21, 1997 pp. 7746-7751
©1997 by The American Society for Biochemistry and Molecular Biology, Inc.

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Cystic Fibrosis Transmembrane Conductance Regulator-associated ATP and Adenosine 3-Phosphate 5-Phosphosulfate Channels in Endoplasmic Reticulum and Plasma Membranes

(Received for publication, January 14, 1997)

From the ^‡ Division of Cell Biology, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada and the ^¶ Department of Physiology and Institute for Human Gene Therapy, University of Pennsylvania, Philadelphia, Pennsylvania 19104-6100

Cystic fibrosis (CF) is characterized by abnormal regulation of epithelial ion and fluid transport due to mutations in the CF transmembrane conductance regulator (CFTR), an apical membrane-localized Cl channel, that usually prevent it from exiting the endoplasmic reticulum. Defective or absent CFTR in the epithelium is believed to disrupt fluid balance in human airways and thereby contribute to chronic respiratory inflammation. Patch-clamp of the plasma membrane and outer membrane of the nuclear envelope of nuclei isolated from CFTR-expressing Chinese hamster ovary cells revealed that CFTR is associated with a regulated ATP channel in both membrane compartments. CFTR expression was also shown to be associated with permeability to another adenine nucleotide, adenosine 3-phosphate 5-phosphosulfate, the universal sulfate donor in cells. These results may provide a link between the ion channel function of CFTR and abnormal glycoprotein processing observed in CF.

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