Cho Do-Yeon, Woodworth Bradford A
Adv Otorhinolaryngol. 2016;79:78-85. doi: 10.1159/000445134. Epub 2016 Jul 28.
In the genetic airway disease cystic fibrosis (CF), deficiency or dysfunction of the cystic fibrosis membrane conductance regulator (CFTR) alters anion transport in respiratory epithelium and consequently disrupts mucociliary clearance. An enriched understanding of the role of CFTR in the maintenance of normal epithelial function has revealed that mild and variable CFTR mutations play a causative role in a number of diseases not classically associated with CF. Furthermore, recent evidence indicates that acquired defects in wild-type CFTR protein processing, endocytic recycling and function can contribute to the pathogenesis of airway diseases, such as chronic obstructive pulmonary disease. In this chapter, we discuss emerging findings implicating acquired CFTR dysfunction in the pathogenesis of chronic rhinosinusitis and propose a new and leading edge approach to future CRS therapy using CFTR potentiators.
在遗传性气道疾病囊性纤维化（CF）中，囊性纤维化跨膜传导调节因子（CFTR）的缺乏或功能障碍会改变呼吸道上皮中的阴离子转运，进而破坏黏液纤毛清除功能。对CFTR在维持正常上皮功能中所起作用的深入了解表明，轻度和可变的CFTR突变在一些并非典型与CF相关的疾病中起致病作用。此外，最近的证据表明，野生型CFTR蛋白加工、内吞再循环和功能方面的后天缺陷可导致气道疾病的发病机制，如慢性阻塞性肺疾病。在本章中，我们讨论了有关后天性CFTR功能障碍在慢性鼻窦炎发病机制中的新发现，并提出了一种使用CFTR增强剂进行未来CRS治疗的新的前沿方法。
