作者
Nam Soo Joo, Toshiya Irokawa, Jin V Wu, Robert C Robbins, Richard I Whyte, Jeffrey J Wine
发表日期
2002/12/27
期刊
Journal of Biological Chemistry
卷号
277
期号
52
页码范围
50710-50715
出版商
Elsevier
简介
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 …
引用总数
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学术搜索中的文章
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