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. No accumulation of PC was seen. Other patients with respiratory disease (not CF) showed normal rates of incorporation of choline into platelet PC. Platelets from heterozygote individuals showed intermediate turnover rates of choline incorporation into PC. The increase in choline incorporation into PC in CF platelets was not due to modified or increased sensitivity to either cAMP or prostaglandin E2. The total amount and the proportions of the major phospholipids in platelets of control and CF individuals were identical. These findings indicate an increased turnover rate of this phospholipid in CF cells rather than an increased net synthesis.
Pediatric Metabolism Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892.
M M
MM
Ulane
J D
JD
Butler
A
A
Peri
L
L
Miele
R E
RE
Ulane
V S
VS
Hubbard
D001483:Base Sequence; D001792:Blood Platelets / Q000187:drug effects / Q000378:metabolism*; D002478:Cells, Cultured; D002794:Choline / Q000378:metabolism; D000242:Cyclic AMP / Q000494:pharmacology; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism*; D017931:DNA Primers; D015232:Dinoprostone / Q000494:pharmacology; D005347:Fibroblasts / Q000378:metabolism; D006579:Heterozygote; D006801:Humans; D008969:Molecular Sequence Data; D010713:Phosphatidylcholines / Q000096:biosynthesis* / Q000097:blood; D016133:Polymerase Chain Reaction; D012016:Reference Values; D012867:Skin / Q000378:metabolism*
D017931:DNA Primers; D010713:Phosphatidylcholines; D000242:Cyclic AMP; D015232:Dinoprostone; D002794:Choline