囊性纤维化的应激源和应激反应

IF 0.7 Cell Pathology Pub Date : 2018-10-01 DOI:10.1515/ersc-2018-0002
Z. Bebok, Lianwu Fu
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引用次数: 4

摘要

囊性纤维化(CF)是由囊性纤维化跨膜传导调节基因(CFTR)突变引起的一种缩短寿命的遗传性疾病。CF的主要原因是CFTR突变导致CFTR介导的氯化物和碳酸氢盐运输减少。然而,炎症和持续感染影响临床结果。细胞应激反应途径,如未折叠蛋白反应(UPR)和综合应激反应(ISR),在这里被称为细胞应激反应途径(SRPs),有助于人类疾病的病理。多项研究表明,CF组织中存在SRPs的活化。我们回顾了目前对CF中SRPs如何被激活及其对病理的贡献的理解。我们的结论是,CF器官中CFTR功能的减少建立了一个组织环境,在这个环境中,内部或外部的损伤激活了srp。SRPs通过降低CFTR的表达,增强炎症和随之而来的组织重塑来促进CF的发病机制。即使在CFTR“调节剂”时代,了解SRPs对CF发病机制的贡献也是至关重要的,CFTR“调节剂”旨在增强、纠正或放大CFTR功能,因为迫切需要支持性治疗。重要的是,病理确定的CF患者可以从靶向使用调节srp的药物中获益,以减轻症状。
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Stressors and Stress Responses in Cystic Fibrosis
Abstract Cystic fibrosis (CF) is a life-shortening, genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). The primary cause of CF is reduced CFTR-mediated chloride and bicarbonate transport, due to mutations in CFTR. However, inflammation and persistent infections influence clinical outcome. Cellular stress response pathways, such as the unfolded protein response (UPR) and the integrated stress response (ISR), referred to here as cellular stress response pathways (SRPs), contribute to the pathology of human disorders. Multiple studies have indicated activation of SRPs in CF tissues. We review our present understanding of how SRPs are activated in CF and their contribution to pathology. We conclude that reduced CFTR function in CF organs establishes a tissue environment in which internal or external insults activate SRPs. SRPs contribute to CF pathogenesis by reducing CFTR expression, enhancing inflammation with consequent tissue remodeling. Understanding the contribution of SRPs to CF pathogenesis is crucial even in the era of CFTR “modulators” that are designed to potentiate, correct or amplify CFTR function, since there is an urgent need for supportive treatments. Importantly, CF patients with established pathology could benefit from the targeted use of drugs that modulate SRPs to reduce the symptoms.
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