CFTR-adenylyl cyclase I association responsible for UTP activation of CFTR in well-differentiated primary human bronchial cell cultures

Mol Biol Cell. 2010 Aug 1;21(15):2639-48. doi: 10.1091/mbc.E09-12-1004. Epub 2010 Jun 16.

Abstract

Chloride secretion by airway epithelial cells is defective in cystic fibrosis (CF). The conventional paradigm is that CFTR is activated through cAMP and protein kinase A (PKA), whereas the Ca(2+)-activated chloride channel (CaCC) is activated by Ca(2+) agonists like UTP. We found that most chloride current elicited by Ca(2+) agonists in primary cultures of human bronchial epithelial cells is mediated by CFTR by a mechanism involving Ca(2+) activation of adenylyl cyclase I (AC1) and cAMP/PKA signaling. Use of selective inhibitors showed that Ca(2+) agonists produced more chloride secretion from CFTR than from CaCC. CFTR-dependent chloride secretion was reduced by PKA inhibition and was absent in CF cell cultures. Ca(2+) agonists produced cAMP elevation, which was blocked by adenylyl cyclase inhibition. AC1, a Ca(2+)/calmodulin-stimulated adenylyl cyclase, colocalized with CFTR in the cell apical membrane. RNAi knockdown of AC1 selectively reduced UTP-induced cAMP elevation and chloride secretion. These results, together with correlations between cAMP and chloride current, suggest that compartmentalized AC1-CFTR association is responsible for Ca(2+)/cAMP cross-talk. We further conclude that CFTR is the principal chloride secretory pathway in non-CF airways for both cAMP and Ca(2+) agonists, providing a novel mechanism to link CFTR dysfunction to CF lung disease.

Publication types

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

MeSH terms

  • Adenylyl Cyclases / metabolism*
  • Bronchi / cytology*
  • Calcium / agonists
  • Calcium / metabolism
  • Cell Differentiation / drug effects*
  • Cell Membrane / drug effects
  • Cell Membrane / metabolism
  • Cell Polarity / drug effects
  • Cells, Cultured
  • Chlorides / metabolism
  • Cyclic AMP / metabolism
  • Cyclic AMP-Dependent Protein Kinases / metabolism
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Electric Conductivity
  • Enzyme Activation / drug effects
  • Epithelial Cells / cytology
  • Epithelial Cells / drug effects
  • Epithelial Cells / enzymology
  • Epithelial Cells / metabolism*
  • Humans
  • Ion Channel Gating / drug effects*
  • Potassium Channels / metabolism
  • Protein Transport / drug effects
  • Signal Transduction / drug effects
  • Uridine Triphosphate / pharmacology*

Substances

  • Chlorides
  • Potassium Channels
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Cyclic AMP
  • Cyclic AMP-Dependent Protein Kinases
  • Adenylyl Cyclases
  • Calcium
  • Uridine Triphosphate