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囊性纤维化基因表达与整流性氯离子通道无关。

Cystic fibrosis gene expression is not correlated with rectifying Cl- channels.

作者信息

Ward C L, Krouse M E, Gruenert D C, Kopito R R, Wine J J

机构信息

Cystic Fibrosis Research Laboratory, Stanford University, CA 94305.

出版信息

Proc Natl Acad Sci U S A. 1991 Jun 15;88(12):5277-81. doi: 10.1073/pnas.88.12.5277.

Abstract

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.

摘要

囊性纤维化(CF)涉及多种外分泌组织中氯离子通透性的显著降低。有人提出一种独特的、外向整流的、去极化诱导的氯离子通道(ORDIC通道)可解释CF中存在缺陷的氯电导。最近鉴定出的CF基因预计编码一种1480个氨基酸的整合膜蛋白,称为CF跨膜电导调节因子(CFTR)。CFTR与ATP结合膜转运蛋白超家族(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。有人提出CFTR可能是一种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中CFTR和ORDIC通道表达的自然变异。在涵盖CFTR mRNA水平和ORDIC通道密度全部观察范围的四个人类上皮细胞系(T84、CaCo2、PANC-1和9HTEo-/S)中,我们未发现相关性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0da/51855/be551ddbdf05/pnas01062-0222-a.jpg

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