Cystic fibrosis gene expression is not correlated with rectifying Cl- channels.
Cystic fibrosis gene expression is not correlated with rectifying Cl- channels.
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囊性纤维化基因表达与纠正 Cl-通道无关。
DOI：
10.1073/pnas.88.12.5277
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发表时间：
1991
影响因子：
11.1
通讯作者：
Wine,JJ
中科院分区：
文献类型：
--
作者：
Ward,CL;Krouse,ME;Gruenert,DC;Kopito,RR;Wine,JJ
囊性纤维化（CF）涉及几种外分泌组织中氯离子渗透性的显着降低。人们提出了一种独特的、向外整流的、去极化诱导的 Cl- 通道（ORDIC 通道）来解释 CF 中存在缺陷的 Cl- 电导。最近发现的 CF 基因预计会编码一种 1480 个氨基酸的整合膜蛋白，称为 CF 跨膜电导调节器 (CFTR)。 CFTR 与 ATP 结合膜转运蛋白超家族（例如 P-糖蛋白和 STE6）具有序列相似性，但它也具有与离子通道功能一致的特征。有人提议 CFTR 可能是一个 ORDIC 通道。为了确定 CFTR 和 ORDIC 通道表达是否相关，我们调查了各种细胞系的 CFTR 和 ORDIC 通道表达的自然变化。在涵盖 CFTR mRNA 水平和 ORDIC 通道密度的完整观察范围的四种人类上皮细胞系（T84、CaCo2、PANC-1 和 9HTEo-/S）中，我们发现没有相关性。
Cystic fibrosis (CF) involves a profound reduction of Cl- permeability in several exocrine tissues. A distinctive, outwardly rectifying, depolarization-induced Cl- channel (ORDIC channel) has been proposed to account for the Cl- conductance that is defective in CF. The recently identified CF gene is predicted to code for a 1480-amino acid integral membrane protein termed the CF transmembrane conductance regulator (CFTR). The CFTR shares sequence similarity with a superfamily of ATP-binding membrane transport proteins such as P-glycoprotein and STE6, but it also has features consistent with an ion channel function. It has been proposed that the CFTR might be an ORDIC channel. To determine if CFTR and ORDIC channel expression are correlated, we surveyed various cell lines for natural variation in CFTR and ORDIC channel expression. In four human epithelial cell lines (T84, CaCo2, PANC-1, and 9HTEo-/S) that encompass the full observed range of CFTR mRNA levels and ORDIC channel density we found no correlation.