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: Contribution to journalArticle

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
Pages (from-to)419-441
Number of pages23
JournalMethods in molecular biology (Clifton, N.J.)
Volume741
DOIs
Publication statusPublished - 2011 Sep 7

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ASJC Scopus subject areas

  • Molecular Biology
  • Genetics

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