Revisiting Host-Pathogen Interactions in Cystic Fibrosis Lungs in the Era of CFTR Modulators.
Revisiting Host-Pathogen Interactions in Cystic Fibrosis Lungs in the Era of CFTR Modulators.
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DOI：
10.3390/ijms24055010
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发表时间：
2023-03-05
影响因子：
5.6
通讯作者：
中科院分区：
文献类型：
--
作者：
囊性纤维化跨膜传导调节剂（CFTR）调节剂是一系列新的治疗药物，可纠正和增强CFTR的某些类型的突变，为囊性纤维化（pwCF）患者提供了巨大的治疗优势。目前CFTR调节剂的主要障碍与它们在减少慢性肺部细菌感染和炎症方面的局限性有关，慢性细菌感染和炎症是肺组织损伤和进行性呼吸功能不全的主要原因，特别是在CF成人中。在这里，我们重新讨论了pwCF中肺部细菌感染和炎症过程中最具争议的问题。特别关注细菌感染pwCF的机制，铜绿假单胞菌的进行性适应及其与金黄色葡萄球菌的相互作用，细菌之间的串扰，支气管上皮细胞和宿主免疫防御的吞噬细胞。本文还介绍了CFTR调节剂对细菌感染和炎症过程的影响的最新发现，为确定相关的治疗靶点以克服pwCF的呼吸病理提供了关键提示。
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators, a new series of therapeutics that correct and potentiate some classes of mutations of the CFTR, have provided a great therapeutic advantage to people with cystic fibrosis (pwCF). The main hindrances of the present CFTR modulators are related to their limitations in reducing chronic lung bacterial infection and inflammation, the main causes of pulmonary tissue damage and progressive respiratory insufficiency, particularly in adults with CF. Here, the most debated issues of the pulmonary bacterial infection and inflammatory processes in pwCF are revisited. Special attention is given to the mechanisms favoring the bacterial infection of pwCF, the progressive adaptation of Pseudomonas aeruginosa and its interplay with Staphylococcus aureus, the cross-talk among bacteria, the bronchial epithelial cells and the phagocytes of the host immune defenses. The most recent findings of the effect of CFTR modulators on bacterial infection and the inflammatory process are also presented to provide critical hints towards the identification of relevant therapeutic targets to overcome the respiratory pathology of pwCF.
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影响因子：
7.3
作者：
Button B;Okada SF;Frederick CB;Thelin WR;Boucher RC
通讯作者：
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DOI：
10.1164/rccm.202002-0369oc
发表时间：
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影响因子：
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影响因子：
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作者：
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影响因子：
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作者：
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