Cystic Fibrosis Lung Immunity: The Role of the Macrophage.

Cystic Fibrosis Lung Immunity: The Role of the Macrophage.
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DOI:
10.1159/000446825
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发表时间:
2016
影响因子:
5.3
通讯作者:
Bonfield TL
Bonfield TL
中科院分区:
医学2区
文献类型:
--
作者:
Bruscia EM;Bonfield TL

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CF 病理生理学的特点是过度炎症和无法有效解决肺部感染,导致患有这种疾病的患者出现严重的病态并最终导致死亡。巨噬细胞通过对先天性和适应性免疫网络的多种贡献而成为肺稳态的主要参与者。 CF 中巨噬细胞功能和活性的设置是多方面的,包括对 CF 肺中独特环境线索的反应,以及 CFTR 功能障碍引起的内在变化。随着修饰基因的鉴定,复杂性进一步增强,修饰基因调节 CFTR 对疾病的贡献,导致巨噬细胞表型的表观遗传和转录变化。本综述重点关注巨噬细胞对肺稳态的贡献,概述了各种文献以及关于这些免疫卫士在 CF 中的作用的不同观点。
CF pathophysiology is hallmarked by excessive inflammation and the inability to efficiently resolve lung infections contributing to major morbidly and eventually the mortality of patients with this disease. Macrophages are major players in lung homeostasis through their diverse contributions to both innate and adaptive immune networks. The setting of macrophage function and activity in CF is multifaceted encompassing response to the unique environmental cues in the CF lung, as well as the intrinsic changes resulting from CFTR dysfunction. The complexity is further enhanced with the identification of modifier genes, which modulate the CFTR contribution to disease resulting in epigenetic and transcriptional shifts in macrophage phenotype. This review focuses on the contribution of macrophages to lung homeostasis overviewing the diverse literature and the varied perspectives on the role of these immune guardians in CF.
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