Duszyk M, French A S, Man S F
Department of Physiology, University of Alberta, Edmonton, Canada.
Can J Physiol Pharmacol. 1989 Oct;67(10):1362-5. doi: 10.1139/y89-217.
Abnormalities of epithelial function in cystic fibrosis (CF) have been linked to defects in cell membrane permeability to chloride or sodium ions. Recently, a class of chloride channels in airway epithelial cells have been reported to lack their usual sensitivity to phosphorylation via cAMP-dependent protein kinase, suggesting that CF could be due to a single genetic defect in these channels. We have examined single chloride and sodium channels in control and CF human nasal epithelia using the patch-clamp technique. The most common chloride channel was not the one previously associated with CF, but it was also abnormal in CF cells. In addition, the number of sodium channels was unusually high in CF. These findings suggest a wider disturbance of ion channel properties in CF than would be produced by a defect in a single type of channel.
囊性纤维化（CF）中上皮功能异常与细胞膜对氯离子或钠离子通透性缺陷有关。最近，有报道称气道上皮细胞中的一类氯离子通道对环磷酸腺苷（cAMP）依赖性蛋白激酶介导的磷酸化缺乏正常敏感性，这表明CF可能是由于这些通道中的单一基因缺陷所致。我们使用膜片钳技术研究了对照和CF患者人鼻上皮细胞中的单个氯离子和钠离子通道。最常见的氯离子通道并非先前与CF相关的那个通道，但在CF细胞中它也是异常的。此外，CF细胞中的钠离子通道数量异常高。这些发现表明，CF中离子通道特性的紊乱比单一类型通道缺陷所产生的紊乱更为广泛。
