Cyclic adenosine monophosphate-dependent kinase in cystic fibrosis tracheal epithelium

R. Barthelson, Jonathan Widdicombe

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16 Scopus citations


Cl- impermeability in cystic fibrosis (CF) tracheal epithelium derives from a deficiency in the β-adrenergic regulation of apical membrane Cl- channels. To test the possibility that cAMP-dependent kinase is the cause of this deficiency, we assayed this kinase in soluble fractions from cultured airway epithelial cells, including CF human tracheal epithelial cells. Varying levels of cAMP were used in these assays to derive both a V(max) and apparent dissociation constant (K(d)) for the enzymes in soluble extracts. The cAMP-dependent protein kinase from CF human tracheal epithelial cells has essentially the same V(max) and apparent K(d) as non-CF human, bovine, and dog tracheal epithelial cells. Thus, the total activity of the cAMP-dependent kinases and their overall responsiveness to cAMP are unchanged in CF.

Original languageEnglish (US)
Pages (from-to)1799-1802
Number of pages4
JournalJournal of Clinical Investigation
Issue number6
StatePublished - 1987
Externally publishedYes

ASJC Scopus subject areas

  • Medicine(all)


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