Application of High-Resolution Single-Channel Recording to Functional Studies of Cystic Fibrosis Mutants

Zhiwei Cai, Yoshiro Sohma, Silvia G. Bompadre, David N. Sheppard, Tzyh Chang Hwang

Research output: Chapter in Book/Report/Conference proceedingChapter

11 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 publicationMethods in Molecular Biology
PublisherHumana Press Inc.
Pages419-441
Number of pages23
DOIs
Publication statusPublished - 2011
Externally publishedYes

Publication series

NameMethods in Molecular Biology
Volume741
ISSN (Print)1064-3745
ISSN (Electronic)1940-6029

Keywords

  • ATP-binding cassette transporter
  • CFTR
  • channel gating
  • chloride ion channel
  • patch-clamp technique
  • single-channel recording

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics

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