Airway Epithelium Dysfunction in Cystic Fibrosis and COPD.
Airway Epithelium Dysfunction in Cystic Fibrosis and COPD.
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DOI：
10.1155/2018/1309746
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发表时间：
2018
影响因子：
4.6
通讯作者：
Greene CM
中科院分区：
文献类型：
--
作者：
De Rose V;Molloy K;Gohy S;Pilette C;Greene CM
囊性纤维化是一种由CFTR基因突变引起的遗传性疾病，而慢性阻塞性肺疾病（COPD）主要由环境因素（主要是吸烟）引起，具有遗传易感背景。虽然这些疾病的病因和发病机制不同，但都与进行性气流阻塞、气道中性粒细胞炎症和反复发作有关，提示其共同的机制。气道上皮在维持正常气道功能中起着至关重要的作用。CF和COPD的气道上皮都发生了主要的分子和形态学变化，越来越多的证据表明，气道上皮功能障碍参与了两种疾病的发病和进展。气道和肺泡上皮的结构和功能异常对宿主防御、免疫/炎症反应和修复过程的改变有相关影响，导致进行性肺损伤和肺功能受损。在这篇综述中，我们讨论了功能失调的气道上皮细胞在CF和COPD的慢性气道炎症和重塑中起关键作用的证据，强调了涉及上皮功能障碍的共同机制以及两种疾病的异同。
Cystic fibrosis is a genetic disease caused by mutations in the CFTR gene, whereas chronic obstructive pulmonary disease (COPD) is mainly caused by environmental factors (mostly cigarette smoking) on a genetically susceptible background. Although the etiology and pathogenesis of these diseases are different, both are associated with progressive airflow obstruction, airway neutrophilic inflammation, and recurrent exacerbations, suggesting common mechanisms. The airway epithelium plays a crucial role in maintaining normal airway functions. Major molecular and morphologic changes occur in the airway epithelium in both CF and COPD, and growing evidence suggests that airway epithelial dysfunction is involved in disease initiation and progression in both diseases. Structural and functional abnormalities in both airway and alveolar epithelium have a relevant impact on alteration of host defences, immune/inflammatory response, and the repair process leading to progressive lung damage and impaired lung function. In this review, we address the evidence for a critical role of dysfunctional airway epithelial cells in chronic airway inflammation and remodelling in CF and COPD, highlighting the common mechanisms involved in the epithelial dysfunction as well as the similarities and differences of the two diseases.
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DOI：
10.1126/science.1223012
发表时间：
2012-08-24
期刊：
Science (New York, N.Y.)
影响因子：
--
作者：
Button B;Cai LH;Ehre C;Kesimer M;Hill DB;Sheehan JK;Boucher RC;Rubinstein M
通讯作者：
Rubinstein M
DOI：
10.1164/rccm.200509-1461oc
发表时间：
2006-07-01
影响因子：
24.7
作者：
Berenson, Charles S.;Wrona, Catherine T.;Sethi, Sanjay
通讯作者：
Sethi, Sanjay
影响因子：
168.9
作者：
Brusselle, Guy G.;Joos, Guy F.;Bracke, Ken R.
通讯作者：
Bracke, Ken R.
影响因子：
6.4
作者：
Berenson, Charles S.;Garlipp, Mary Alice;Sethi, Sanjay
通讯作者：
Sethi, Sanjay
影响因子：
12.4
作者：
Bogefors, J.;Rydberg, C.;Cardell, L. O.
通讯作者：
Cardell, L. O.