Cystic fibrosis and phosphatidylcholine biosynthesis.

The cystic fibrosis (CF) gene defect may be associated with a defect in membrane recycling. We have investigated the metabolism of the main constituent of plasma membrane, phosphatidylcholine (PC). In this study of platelets and fibroblasts, we show an increased uptake of choline into PC of CF cells as compared with normal cells.

Hormone-stimulated cyclic AMP production by skin fibroblasts cultured from healthy persons and patients with cystic fibrosis.

The experiments reported here illustrate a few of the factors apart from genes which can influence hormone-responsive generation of cyclic adenosine 3':5'-monophosphate in human fibroblasts. For both normal and cystic fibrosis fibroblasts, the isoproterenol stimulation ratio was maximal 2 to 3 days after subculture and declined thereafter; prostaglandin E1 stimulation ratio was maximal 7 to 10 days after subculture. Cells dislodged from the plate by either scraping or typsinization had reduced isoproterenol or prostaglandin E1 stimulation ratios compared to cells studied in situ.