Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid
Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid
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DOI：
10.1165/ajrcmb.25.1.4436
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发表时间：
2001-07-01
影响因子：
6.4
通讯作者：
Wilson, JM
中科院分区：
文献类型：
--
作者：
Bals, R;Weiner, DJ;Wilson, JM
囊性纤维化（CF）中的遗传缺陷与最近描述的肺宿主防御中的突破之间的联系集中在气道中的盐和水代谢的作用上。使用人支气管异种移植物模型，我们证明了CF气道表面液（ASF）中细菌杀灭的盐非依赖性异常。生物化学表征暗示了对正常细菌杀伤的构成至关重要的分子的缺失或功能障碍。我们的研究表明，CF跨膜传导调节因子（CFTR）的缺陷导致的主要异常组成的ASF，导致盐非依赖性的缺陷，在主机的防御。重要的是，这种缺陷通过腺病毒介导的CFTR基因转移来纠正。
The link between the genetic defect in cystic fibrosis (CF) and the recently described breach in pulmonary host defense has focused on the role of salt and water metabolism in the airways. Using a human bronchial xenograft model we demonstrate a salt-independent abnormality in bacterial killing in CF airway surface fluid (ASF). Biochemical characterization implicates the absence or dysfunction of a molecule critical to the constitution of normal bacterial killing. Our study suggests that CF transmembrane conductance regulator (CFTR) deficiency causes a primary abnormality in the composition of ASF that leads to a salt-independent defect in host defense. Importantly, this defect is corrected by adenovirus-mediated gene transfer of CFTR.