PDF(Pubmed)
Abstract：
The epithelia that line the conducting airways are the lung\'s first point of contact with inhaled pathogens and toxicants. As such, they are known to play an important role in the lung\'s innate defense system, which includes (i) the production of airway surface liquid (ASL) that helps cleanse the airways through the physical removal of pathogens and toxicants on the mucociliary escalator and (ii) the secretion of anti-microbial proteins into the ASL to kill inhaled pathogens. Interestingly, the recently crystallized short palate lung and nasal epithelial clone 1 (SPLUNC1) protein appears to be a multi-functional protein. That is, it not only acts as an anti-microbial agent, but also modulates ASL homeostasis by acting as an endogenous inhibitor of the epithelial Na(+) channel (ENaC). This review will focus on the latter function of SPLUNC1, and will discuss new structural and physiological data regarding SPLUNC1\'s failure to function as a regulator of ASL hydration in CF airways.
摘要：
传导气道的上皮是肺部与吸入病原体和毒物的第一接触点。因此，众所周知，它们在肺的先天防御系统中起着重要作用，其包括(i)通过物理去除粘膜纤毛自动扶梯上的病原体和毒物来帮助清洁气道的气道表面液体(ASL)的产生和(ii)将抗微生物蛋白分泌到ASL中以杀死吸入的病原体。有趣的是,最近结晶的短腭肺和鼻上皮克隆1（SPLUNC1）蛋白似乎是一种多功能蛋白。也就是说,它不仅是一种抗微生物剂，但也通过作为上皮Na（+）通道（ENaC）的内源性抑制剂来调节ASL稳态。本综述将重点关注SPLUNC1的后一种功能，并将讨论有关SPLUNC1未能作为CF气道中ASL水合调节剂的新结构和生理数据。
