Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue

Am J Respir Cell Mol Biol. 2010 Nov;43(5):607-16. doi: 10.1165/rcmb.2009-0281OC. Epub 2009 Dec 30.

Abstract

Therapies to correct the ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) folding defect require sensitive methods to detect channel activity in vivo. The β₂ adrenergic receptor agonists, which provide the CFTR stimuli commonly used in nasal potential difference assays, may not overcome the channel gating defects seen in ΔF508 CFTR after plasma membrane localization. In this study, we identify an agent, quercetin, that enhances the detection of surface ΔF508 CFTR, and is suitable for nasal perfusion. A screen of flavonoids in CFBE41o⁻ cells stably transduced with ΔF508 CFTR, corrected to the cell surface with low temperature growth, revealed that quercetin stimulated an increase in the short-circuit current. This increase was dose-dependent in both Fisher rat thyroid and CFBE41o⁻ cells. High concentrations inhibited Cl⁻ conductance. In CFBE41o⁻ airway cells, quercetin (20 μg/ml) activated ΔF508 CFTR, whereas the β₂ adrenergic receptor agonist isoproterenol did not. Quercetin had limited effects on cAMP levels, but did not produce detectable phosphorylation of the isolated CFTR R-domain, suggesting an activation independent of channel phosphorylation. When perfused in the nares of Cftr(+) mice, quercetin (20 μg/ml) produced a hyperpolarization of the potential difference that was absent in Cftr(-/-) mice. Finally, quercetin-induced, dose-dependent hyperpolarization of the nasal potential difference was also seen in normal human subjects. Quercetin activates CFTR-mediated anion transport in respiratory epithelia in vitro and in vivo, and may be useful in studies intended to detect the rescue of ΔF508 CFTR by nasal potential difference.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Biomarkers / metabolism*
  • Cell Membrane / drug effects
  • Cell Membrane / metabolism
  • Chlorides / metabolism
  • Cyclic AMP / metabolism
  • Cystic Fibrosis Transmembrane Conductance Regulator / chemistry
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Dose-Response Relationship, Drug
  • Drug Evaluation, Preclinical
  • Humans
  • Ion Channel Gating / drug effects*
  • Ion Transport / drug effects
  • Membrane Potentials / drug effects
  • Mice
  • Mutant Proteins / chemistry
  • Mutant Proteins / metabolism*
  • NIH 3T3 Cells
  • Nasal Mucosa / drug effects
  • Nasal Mucosa / metabolism
  • Phosphorylation / drug effects
  • Protein Structure, Tertiary
  • Quercetin / pharmacology*
  • Rats
  • Receptors, Adrenergic, beta-2 / metabolism

Substances

  • Biomarkers
  • Chlorides
  • Mutant Proteins
  • Receptors, Adrenergic, beta-2
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Quercetin
  • Cyclic AMP