Key result
Chloride channels from cystic fibrosis patients could not be activated by either cAMP-dependent protein kinase or protein kinase C, unlike normal epithelial chloride channels.
Why the study?
Does exposure to cAMP-dependent protein kinase and protein kinase C activate chloride channels in airway epithelial cells from patients with cystic fibrosis compared to normal cells?
Does exposure to cAMP-dependent protein kinase and protein kinase C activate chloride channels in airway epithelial cells from patients with cystic fibrosis compared to normal cells?
The study demonstrates that the gating of epithelial chloride channels by both cAMP-dependent protein kinase and protein kinase C is defective in cystic fibrosis.
Cautions against kinase-based activation strategies in CF now; leaves open targeted research on channel gating defects.
Secretory chloride channels can be activated by adenosine 3',5'-monophosphate (cAMP)-dependent protein kinase in normal airway epithelial cells but not in cells from individuals with cystic fibrosis (CF). In excised, inside-out patches of apical membrane of normal human airway cells and airway cells from three patients with CF, the chloride channels exhibited a characteristic outwardly rectifying current-voltage relation and depolarization-induced activation. Channels from normal tissues were activated by both cAMP-dependent protein kinase and protein kinase C. However, chloride channels from CF patients could not be activated by either kinase. Thus, gating of normal epithelial chloride channels is regulated by both cAMP-dependent protein kinase and protein kinase C, and regulation by both kinases is defective in CF.
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Hwang et al. (1989) studied Cystic Fibrosis (n=3). cAMP-dependent protein kinase and protein kinase C vs. Normal human airway cells was evaluated on Activation of secretory chloride channels. Chloride channels from cystic fibrosis patients could not be activated by either cAMP-dependent protein kinase or protein kinase C, unlike normal epithelial chloride channels.
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