Abnormal levels of 3':5'-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis.

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Abstract
To determine if the abnormalities of exocrine secretion characteristic of cystic fibrosis [CF] could be investigated in vitro, the synthesis of cyclic[c]AMP after isoproterenol stimulation in skin fibroblasts derived from patients with CF and from normal individuals was studied. Comparison of normal and CF cells showed that the latter had 2- to 5-fold greater levels of intracellular cAMP after stimulation with isoproterenol. The difference between the strains was observed at every stage of the culture cycle and was specific to stimulation by .beta.-adrenergic agonists. It could not be accounted for by different dose- or time-response curves nor by leakage of cAMP into the medium. The increased sensitivity to catecholamines may reflect an intrinsic genetic property of CF cells, and it may be feasible to use this system to study the biochemical basis of the genetic defect in CF.