Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.
Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.
复制标题
CFTR 氯离子通道的丧失会改变体外囊性纤维化气道上皮对盐的吸收。
DOI：
10.1016/s1097-2765(00)80284-1
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发表时间：
1998
期刊：
影响因子：
16
通讯作者：
Welsh,MJ
中科院分区：
文献类型：
--
作者：
Zabner,J;Smith,JJ;Karp,PH;Widdicombe,JH;Welsh,MJ
囊性纤维化（CF）是由功能性CFTR Cl−通道的丧失引起的。然而，尚不清楚该缺陷如何破坏气道中的盐和液体运动，或者它是否改变了覆盖气道表面的薄液膜中的NaCl浓度。使用一种新的方法，我们发现CF气道表面液体的NaCl浓度高于正常。CF和非CF上皮细胞都吸收盐和液体;然而，CFTR Cl−通道的表达是最大吸收所必需的。因此，CFTR的丧失通过相关机制提高了CF气道表面液体和汗液中的盐浓度; NaCl浓度升高是由于跨细胞Cl−运动受阻。高NaCl可能通过抑制内源性抗菌防御而使CF气道易受细菌感染。
Cystic fibrosis (CF) is caused by the loss of functional CFTR Cl−channels. However, it is not understood how this defect disrupts salt and liquid movement in the airway or whether it alters the NaCl concentration in the thin liquid film covering the airway surface. Using a new approach, we found that CF airway surface liquid had a higher NaCl concentration than normal. Both CF and non-CF epithelia absorbed salt and liquid; however, expression of CFTR Cl−channels was required for maximal absorption. Thus, loss of CFTR elevates the salt concentration in CF airway surface liquid and in sweat by related mechanisms; the elevated NaCl concentration is due to a block in transcellular Cl−movement. The high NaCl may predispose CF airways to bacterial infections by inhibiting endogenous antibacterial defenses.