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用于评估囊性纤维化跨膜传导调节因子(CFTR)离子通道活性的鼻电位差测量

Nasal potential difference measurements to assess CFTR ion channel activity.

作者信息

Rowe Steven M, Clancy John Paul, Wilschanski Michael

机构信息

Department of Medicine, University of Alabama, Birmingham, AL 35294-0006, USA.

出版信息

Methods Mol Biol. 2011;741:69-86. doi: 10.1007/978-1-61779-117-8_6.

Abstract

The Nasal potential difference measurement is used to measure the voltage across the nasal epithelium, which results from transepithelial ion transport and reflects in part CFTR function. The electrophysiologic abnormality in cystic fibrosis was first described 30 years ago and correlates with features of the CF phenotype. NPD measurement is an important in vivo research and diagnostic tool, and is used to assess the efficacy of new treatments such as gene therapy and ion transport modulators. This chapter will elaborate on the electrophysiological principles behind the test, the equipment required, the methods, and the analysis of the data.

摘要

鼻电位差测量用于测量鼻上皮细胞两端的电压,该电压由跨上皮离子转运产生,部分反映了囊性纤维化跨膜传导调节因子(CFTR)的功能。囊性纤维化的电生理异常在30年前首次被描述,与CF表型的特征相关。鼻电位差测量是一种重要的体内研究和诊断工具,用于评估基因治疗和离子转运调节剂等新治疗方法的疗效。本章将详细阐述该测试背后的电生理原理、所需设备、方法以及数据分析。

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