PMID: 7513123Mar 1, 1994Paper

Differential acidic pH sensitivity of delta F508 CFTR Cl- channel activity in lipid bilayers

The American Journal of Physiology
A M SherryD H Malinowska

Abstract

Cystic fibrosis transmembrane conductance regulator (CFTR) is present in acidic intracellular vesicles. Human normal and delta F508 CFTR Cl- channel characteristics at pH 7.4 and pH 4.5 were determined by fusing Xenopus laevis oocyte plasma membranes containing the expressed channels to planar lipid bilayers. At pH 7.4, both channels exhibited linear current-voltage curves, a 10 +/- 0.3-pS conductance using 800 mM CsCl, and a 9:1 Cl-/Cs+ discrimination ratio obtained from a 32 +/- 2 mV reversal potential with a fivefold gradient. At -80 mV, the open probability (Po) of mutant CFTR was 53% that of normal CFTR. Reduction of the trans-pH from 7.4 to 4.5 had no effect on the above characteristics except for Po, where it caused a 47% reduction in normal CFTR Po (due to a 75% decrease in mean open time) and a 75% reduction in delta F508 CFTR Po (due to a 6-fold increase in mean closed time). Normal CFTR can thus function in the environment of acidic intracellular organelles, whereas activity of mutant CFTR would be greatly reduced. These results may be of significance to understanding the cystic fibrosis defect.

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Citations

Oct 1, 1998·The American Journal of Physiology·M M ReddyP M Quinton
Oct 27, 2010·The Journal of Biological Chemistry·Raffaella MelaniOlga Zegarra-Moran
May 24, 2013·Adipocyte·Michelle T Foster, Michael J Pagliassotti
Aug 1, 1995·Nature Genetics·W H ColledgeM J Evans
Jun 1, 2004·Experimental Biology and Medicine·Dorothy B HausmanRuth B S Harris
May 10, 2007·Canadian Journal of Physiology and Pharmacology·Yi-Ping HouGui-Lin Xie
Apr 27, 2007·Journal of Lipid Research·Timothy J Bartness, C K Song
Nov 17, 2001·Clinical Autonomic Research : Official Journal of the Clinical Autonomic Research Society·M Barczyński, P Thor
May 11, 2012·American Journal of Physiology. Cell Physiology·Ji LiuClaire H Mitchell
Aug 29, 2007·The Journal of Biological Chemistry·Peter M Haggie, A S Verkman
Feb 7, 2004·Neurogastroenterology and Motility : the Official Journal of the European Gastrointestinal Motility Society·M J MonroeE R Partosoedarso
May 22, 1996·Biochimica Et Biophysica Acta·P HuangG A Scarborough

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