Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants

Z Cai, Y Sohma, SG Bompadre, DN Sheppard, TC Hwang

Research output: Chapter in Book/Report/Conference proceedingChapter in a book

5 Citations (Scopus)

Abstract

The patch-clamp technique is a powerful and versatile method to investigate the cystic fibrosis transmembrane conductance regulator (CFTR) Cl– channel, its malfunction in disease and modulation by small molecules. Here, we discuss how the molecular behaviour of CFTR is investigated using high-resolution single-channel recording and kinetic analyses of channel gating. We review methods used to quantify how cystic fibrosis (CF) mutants perturb the biophysical properties and regulation of CFTR. By explaining the relationship between macroscopic and single-channel currents, we demonstrate how single-channel data provide molecular explanations for changes in CFTR-mediated transepithelial ion transport elicited by CF mutants.
Original languageEnglish
Title of host publicationCystic Fibrosis Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects, Methods in Molecular Biology
EditorsMD Amaral, K Kunzelmann
PublisherHumana Press
Pages419 - 441
Number of pages22
Volume741
ISBN (Print)9791617791161
Publication statusPublished - 2011

Bibliographical note

Other identifier: 10.1007/978-1-61779-117-8_27

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  • Cite this

    Cai, Z., Sohma, Y., Bompadre, SG., Sheppard, DN., & Hwang, TC. (2011). Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants. In MD. Amaral, & K. Kunzelmann (Eds.), Cystic Fibrosis Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects, Methods in Molecular Biology (Vol. 741, pp. 419 - 441). Humana Press.