Literature DB >> 21594800

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

Zhiwei Cai1, Yoshiro Sohma, Silvia G Bompadre, David N Sheppard, Tzyh-Chang Hwang.   

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.

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Year:  2011        PMID： 21594800      PMCID： PMC4939242          DOI： 10.1007/978-1-61779-117-8_27

Source DB:  PubMed          Journal:  Methods Mol Biol        ISSN： 1064-3745


  59 in total

Review 1.  Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by its R domain.

Authors:  L S Ostedgaard; O Baldursson; M J Welsh
Journal:  J Biol Chem       Date:  2001-01-23       Impact factor: 5.157

2.  Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation.

Authors:  W Dalemans; P Barbry; G Champigny; S Jallat; K Dott; D Dreyer; R G Crystal; A Pavirani; J P Lecocq; M Lazdunski
Journal:  Nature       Date:  1991 Dec 19-26       Impact factor: 49.962

3.  Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.

Authors:  M L Drumm; D J Wilkinson; L S Smit; R T Worrell; T V Strong; R A Frizzell; D C Dawson; F S Collins
Journal:  Science       Date:  1991-12-20       Impact factor: 47.728

4.  Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity.

Authors:  Zhiwei Cai; David N Sheppard
Journal:  J Biol Chem       Date:  2002-03-19       Impact factor: 5.157

5.  Dual effects of ADP and adenylylimidodiphosphate on CFTR channel kinetics show binding to two different nucleotide binding sites.

Authors:  F Weinreich; J R Riordan; G Nagel
Journal:  J Gen Physiol       Date:  1999-07       Impact factor: 4.086

6.  Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches.

Authors:  O P Hamill; A Marty; E Neher; B Sakmann; F J Sigworth
Journal:  Pflugers Arch       Date:  1981-08       Impact factor: 3.657

7.  Phosphorylation-regulated Cl- channel in CHO cells stably expressing the cystic fibrosis gene.

Authors:  J A Tabcharani; X B Chang; J R Riordan; J W Hanrahan
Journal:  Nature       Date:  1991-08-15       Impact factor: 49.962

8.  Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis. Quantitative analysis of a cyclic gating scheme.

Authors:  S Zeltwanger; F Wang; G T Wang; K D Gillis; T C Hwang
Journal:  J Gen Physiol       Date:  1999-04       Impact factor: 4.086

9.  Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator.

Authors:  M H Akabas; C Kaufmann; T A Cook; P Archdeacon
Journal:  J Biol Chem       Date:  1994-05-27       Impact factor: 5.157

10.  CFTR gating I: Characterization of the ATP-dependent gating of a phosphorylation-independent CFTR channel (DeltaR-CFTR).

Authors:  Silvia G Bompadre; Tomohiko Ai; Jeong Han Cho; Xiaohui Wang; Yoshiro Sohma; Min Li; Tzyh-Chang Hwang
Journal:  J Gen Physiol       Date:  2005-03-14       Impact factor: 4.086
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  6 in total

1.  Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.

Authors:  Wen-Ying Lin; Yoshiro Sohma; Tzyh-Chang Hwang
Journal:  Mol Pharmacol       Date:  2016-07-13       Impact factor: 4.436

2.  Functional reconstitution and channel activity measurements of purified wildtype and mutant CFTR protein.

Authors:  Paul D W Eckford; Canhui Li; Christine E Bear
Journal:  J Vis Exp       Date:  2015-03-09       Impact factor: 1.355

Review 3.  The gating of the CFTR channel.

Authors:  Oscar Moran
Journal:  Cell Mol Life Sci       Date:  2016-10-01       Impact factor: 9.261

Review 4.  Assays of CFTR Function In Vitro, Ex Vivo and In Vivo.

Authors:  Anabela Santo Ramalho; Mieke Boon; Marijke Proesmans; François Vermeulen; Marianne S Carlon; Kris De Boeck
Journal:  Int J Mol Sci       Date:  2022-01-27       Impact factor: 5.923

5.  CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.

Authors:  Karen S Raraigh; Kathleen C Paul; Jennifer L Goralski; Erin N Worthington; Anna V Faino; Stanley Sciortino; Yiting Wang; Melis A Aksit; Hua Ling; Derek L Osorio; Frankline M Onchiri; Shivani U Patel; Christian A Merlo; Kristina Montemayor; Ronald L Gibson; Natalie E West; Amita Thakerar; Robert J Bridges; David N Sheppard; Neeraj Sharma; Garry R Cutting
Journal:  JCI Insight       Date:  2022-03-22

6.  A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR.

Authors:  Jia Liu; Allison P Berg; Yiting Wang; Walailak Jantarajit; Katy J Sutcliffe; Edward B Stevens; Lishuang Cao; Marko J Pregel; David N Sheppard
Journal:  Br J Pharmacol       Date:  2022-01-21       Impact factor: 9.473
  6 in total

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