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J. Biol. Chem., Vol. 277, Issue 52, 50710-50715, December 27, 2002
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From the We are testing the hypothesis that the
malfunctioning of airway gland serous cells is a component of cystic
fibrosis (CF) airway disease. CF is caused by mutations that disrupt CF
transmembrane conductance regulator, an anion channel essential
for proper fluid secretion in some epithelia. Submucosal glands
supply most of the mucus in upper airways, and gland serous cells are
the primary site of CF transmembrane conductance regulator expression
in airways. We have discovered a major defect in CF glands by in
situ optical monitoring of secretions from single human airway
glands. CF glands did not secrete to agents that elevated
[cAMP]i (0 responses/450 glands, 8 subjects), whereas glands
were responsive in all donor tracheas (605/827 glands, 15 subjects) and
in bronchi from subjects who were transplanted because of other lung
diseases (148/166 glands, n = 10). CF glands secreted
to cholinergic stimulation, and serous cells were abundant in glands
from all CF subjects. The complete absence of secretion to agents that
elevate [cAMP]i suggests that altered secretion of gland
mucus could contribute to CF lung disease.
Absent Secretion to Vasoactive Intestinal Peptide
in Cystic Fibrosis Airway Glands*
,,,¶
Cystic Fibrosis Research Laboratory,
Stanford University, Stanford, California 94305-2130 and the
§ Cardiothoracic Surgery and School of Medicine, Stanford
University, Stanford, California 94305-5407
*
This work was supported by National Institutes of Health
Grants DK-51817 and HL-60288 and by the Cystic Fibrosis Foundation.The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in
accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
Copyright © 2002 by The American Society for Biochemistry and Molecular Biology, Inc.
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