Reversed anion selectivity in cultured cystic fibrosis sweat duct cells.
Reversed anion selectivity in cultured cystic fibrosis sweat duct cells.
复制标题
DOI：
10.1152/ajpcell.1992.262.1.c32
复制
发表时间：
1992
期刊：
影响因子：
--
通讯作者：
C. L. Bell;M. Reddy;Paul M. Quinton
中科院分区：
文献类型：
--
作者：
C. L. Bell;M. Reddy;Paul M. Quinton
囊性纤维化（CF）是一种以上皮细胞氯离子传导（GCl）缺陷为特征的人类遗传性疾病.为了将CF影响的GCl与其他Cl-通道区分开来，我们研究了正常和CF细胞中GCl的特性，CF细胞生长自吸收性重吸收性汗管（RD）。正常人的培养细胞保留了一些典型的导管细胞特性。Na+传导抑制剂阿米洛利使细胞内电位（Vm）超极化10.4 +/- 1.6 mV（n = 12）。葡萄糖酸盐取代Cl-去极化Vm 15.5 +/- 1.1 mV（n = 33）。正常细胞的表观GCl（G 'Cl）对3'，5 '-环一磷酸腺苷（毛喉素，10（-6）M）敏感，这一点可以通过Cl-梯度诱导的去极化显着增加（63%，n = 9）得到证明，并且对Cl-比I-更具选择性（I-去极化Vm取代Cl-6.3 +/- 0.3 mV，n = 49）。尽管CF受试者的细胞与正常细胞基于Vm（-18.5 +/-1.2mV，n = 49 vs. -20.1 +/-1.8mV，n = 28）在统计学上无法区分，但CF细胞在G’Cl、对毛喉素的反应和阴离子选择性方面表达差异。CF细胞具有显著降低的G’Cl，如通过对施加的Cl-梯度的钝化反应所指示的（正常的26%，n = 28）。与我们在正常细胞中的观察相反，CF细胞的G 'Cl对forskolin不敏感。（250字处删节）
The human genetic disease cystic fibrosis (CF) is characterized by defective epithelial Cl- conductance (GCl). To distinguish the CF-affected GCl from other Cl- channels, we have studied the properties of GCl in normal and CF cells grown from explanted reabsorptive sweat ducts (RD). The cultured cells from normal subjects retained some of the typical duct cell properties. The Na+ conductance inhibitor amiloride hyperpolarized intracellular potentials (Vm) by 10.4 +/- 1.6 mV (n = 12). Substitution of gluconate for Cl- depolarized Vm by 15.5 +/- 1.1 mV (n = 33). The apparent GCl (G'Cl) of normal cells was sensitive to adenosine 3',5'-cyclic monophosphate (forskolin, 10(-6) M), as evidenced by a significant increase (63%, n = 9) in the Cl- gradient induced depolarization, and more selective for Cl- than I- (substitution of Cl- by I- depolarized Vm by 6.3 +/- 0.3 mV, n = 49). Although the cells from CF subjects were statistically indistinguishable from normal cells based on Vm (-18.5 +/- 1.2 mV, n = 49 vs. -20.1 +/- 1.8 mV, n = 28), CF cells expressed differences in G'Cl, responses to forskolin, and anion selectivity. CF cells had a significantly reduced G'Cl as indicated by blunted responses to imposed Cl- gradients (26% of normal, n = 28). In contrast to our observations in normal cells, the G'Cl of CF cells was insensitive to forskolin.(ABSTRACT TRUNCATED AT 250 WORDS)