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囊性纤维化患者培养的鼻上皮细胞顶端膜中的非选择性阳离子通道和功能失调的氯离子通道。

Non-selective cation and dysfunctional chloride channels in the apical membrane of nasal epithelial cells cultured from cystic fibrosis patients.

作者信息

Jorissen M, Vereecke J, Carmeliet E, Van den Berghe H, Cassiman J J

机构信息

Center for Human Genetics, University of Leuven, Belgium.

出版信息

Biochim Biophys Acta. 1990 Nov 14;1096(1):52-9. doi: 10.1016/0925-4439(90)90012-e.

Abstract

Chloride channels and non-selective cation channels in the apical membranes of cultured nasal epithelial cells from three cystic fibrosis patients were investigated with the patch-clamp technique. Outwardly rectifying chloride channels were found in 31% of the inside-out patches, but activity of this channel was never observed in cell-attached patches, even after stimulation with adrenaline. In 30% of the patches with chloride channels, activation occurred immediately after excision. Most of the channels, however, activated only after a membrane depolarization of +40 to +120 mV. Once activated, the chloride channels were indistinguishable from those in nasal epithelial cells of control patients. Amiloride-insensitive, calcium- and voltage-dependent, non-selective cation channels were present in 11% of the cell-attached and 43% of the cell-free patches and could not be distinguished from those in controls. The cystic fibrosis chloride channel defect is conserved in cultured nasal epithelial cells, while a non-selective cation channel is apparently not affected.

摘要

采用膜片钳技术对3例囊性纤维化患者培养的鼻上皮细胞顶端膜中的氯离子通道和非选择性阳离子通道进行了研究。在31%的内面向外膜片中发现了外向整流氯离子通道,但即使在用肾上腺素刺激后,在细胞贴附膜片中也从未观察到该通道的活性。在30%有氯离子通道的膜片中,通道在切除后立即激活。然而,大多数通道仅在膜去极化至+40至+120 mV后才激活。一旦激活,氯离子通道与对照患者鼻上皮细胞中的通道无法区分。在11%的细胞贴附膜片和43%的无细胞膜片中存在amiloride不敏感、钙和电压依赖性的非选择性阳离子通道,且与对照中的通道无法区分。囊性纤维化氯离子通道缺陷在培养的鼻上皮细胞中是保守的,而非选择性阳离子通道显然未受影响。

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