American Journal of Physiology-Cell Physiology · 1997 · 100 citations · 16 references
Protein SecretionCytoskeletonCellular PhysiologySignaling PathwayEpithelial Sodium ChannelsMembrane TransportNa+ TransportCell SignalingCell PhysiologyShort Actin FilamentsMolecular PhysiologyBiochemistryIon ChannelsMembrane BiologyCell BiologySignal TransductionNatural SciencesPhysiologyCell MotilityIntracellular TraffickingCellular BiochemistryMedicine
Cystic fibrosis (CF) airway epithelia exhibit enhanced Na+ reabsorption in parallel with diminished Cl- secretion. We tested the hypothesis that actin plays a role in the regulation of a cloned epithelial Na+ channel (ENaC) by the cystic fibrosis transmembrane conductance regulator (CFTR). We found that immunopurified bovine tracheal CFTR coreconstituted into a planar lipid bilayer with alpha,beta,gamma-rat ENaC (rENaC) decreased single-channel open probability (Po) of rENaC in the presence of actin by over 60%, a significantly greater effect than was observed in the absence of actin (approximately 20%). In the presence of actin, protein kinase A plus ATP activated both CFTR and rENaC, but CFTR was activated in a sustained manner, whereas the activation of rENaC was transitory. ATP alone could also activate ENaC transiently in the presence ofactin but had no effect on CFTR. Stabilizing short actin filaments at a fixed length with gelsolin (at a ratio to actin of 2:1) produced a sustained activation of alpha,beta,gamma-rENaC in both the presence or absence of CFTR. Gelsolin alone (i.e., in the absence of actin) had no effect on the conductance or Po of either CFTR or rENaC. We have also found that short actin filaments produced their modulatory action on alpha-rENaC independent of the presence of the beta- or gamma-rENaC subunits. In contrast, CFTR did not affect any properties of the channel formed by alpha-rENaC alone, i.e., in the absence of beta- or gamma-rENaC. These results indicate that CFTR can directly downregulate single Na+ channel activity, which may account for the observed differences between Na+ transport in normal and CF-affected airway epithelia. Moreover, the presence of actin confers an enhanced modulatory ability of CFTR on Na+ channels.
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Early death due to defective neonatal lung liquid clearance in αENaC-deficient mice
Edith Hümmler, Pierre Barker, John T. Gatzy et al. · Nature Genetics · 1996 · 876 citations · Full text
Richard C. Boucher, M. Jackson Stutts, Michael R. Knowles et al. · Journal of Clinical Investigation · 1986 · 559 citations · Full text
Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium
Michael R. Knowles, M. Jackson Stutts, Alexander Spock et al. · Science · 1983 · 488 citations
Fibrosis, Hyperpolarization (Biology), Sodium Homeostasis +10