CFTR 和 ENaC 之争：ENaC 在 CF 肺病中的作用有多大？
The CFTR and ENaC debate: how important is ENaC in CF lung disease?
机构信息
Department of Cell, Developmental and Integrative Biology, School of Medicine, University of Alabama at Birmingham, 35294-0005, USA.
出版信息
Am J Physiol Lung Cell Mol Physiol. 2012 Jun 1;302(11):L1141-6. doi: 10.1152/ajplung.00036.2012. Epub 2012 Apr 6.
Abstract
Cystic fibrosis (CF) is caused by the loss of the cystic fibrosis transmembrane conductance regulator (CFTR) function and results in a respiratory phenotype that is characterized by dehydrated mucus and bacterial infections that affect CF patients throughout their lives. Much of the morbidity and mortality in CF results from a failure to clear bacteria from the lungs. What causes the defect in the bacterial clearance in the CF lung has been the subject of an ongoing debate. Here we discuss the arguments for and against the role of the epithelial sodium channel, ENaC, in the development of CF lung disease.
摘要
囊性纤维化（CF）是由囊性纤维化跨膜电导调节因子（CFTR）功能丧失引起的，导致呼吸系统表型，其特征是粘液脱水和细菌感染，影响 CF 患者的一生。CF 患者的大部分发病率和死亡率是由于无法从肺部清除细菌。导致 CF 肺部清除细菌缺陷的原因一直是一个持续争论的问题。在这里，我们讨论了上皮钠通道（ENaC）在 CF 肺部疾病发展中的作用的正反两方面论点。
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