Airway epithelial cells鈥擧yperabsorption in CF?

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• 作者：Karl Kunzelmann ; ^{karl.kunzelmann@vkl.uni-regensburg.de} ; Rainer Schreiber
• 关键词：Cystic fibrosis ; ENaC ; Na+ hyperabsorption ; Airways ; CFTR
• 刊名：The International Journal of Biochemistry and Cell Biology
• 出版年：2012
• 期刊代码：127_13572725
• 类别：med
• 出版时间：August, 2012
• 卷：44
• 期：8
• 页码：1232-1235
• 文件大小：680 K

摘要

Airway epithelial cells transport electrolytes and are central to the disease cystic fibrosis (CF), which is an inherited transport defect affecting smaller airways and a number of other epithelial organs. Clinically, CF is dominated by a chronic lung disease, the main cause of morbidity and mortality. Airway obstruction by thick mucus and chronic infection by Pseudomonas aeruginosa eventually lead to loss of pulmonary function. Loss of function of CFTR Cl^{鈭?/sup> channels was found to be the cause for CF. However, intensive research on the detailed mechanism of CF lung disease for more than 25 years produced a bewildering number of hypotheses and an endless discussion whether reduced Cl鈭?/sup> secretion, primarily located in airway submucosal glands, or dehydration of the airways, driven by a hyperabsorption of Na+ ions, is the primary cause of the disease. Recent results suggest a fine-tuned regulation of the airway fluid layer, but how significant really are Cl鈭?/sup> and Na+ transport?}