Abstract
Diploid skin fibroblasts derived from several unrelated patients with cystic fibrosis (CF) were tested for resistance to the cytotoxic effects of ouabain in comparison with cells from normal individuals. Cells from CF and normal individuals were plated at low density and exposed to ouabain at different concentrations for 24 hr. Dose-response curves showed that CF cells survived significantly better than did normal cells at all ouabain concentrations from 0.1 nM to 1 micronM in potassium-deficient medium. In medium containing the usual amount of potassium (6.25 mM), survival of the CF cells exposed to ouabain was decreased to that of the normal cells. If the glucose concentration of the potassium-deficient medium was decreased to 10% of the usual amount, the resistance of the CF cells to ouabain disappeared and their survival was identical to that of the normal cells. These findings may be useful in understanding the biochemical basis of CG and its clinical manifestations.
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Selected References
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- Barranco S. C., Bolton W. E., Haenelt B. R., Abell C. W. Differences in the incorporation of thymidine into DNA of normal and cystic fibrosis fibroblasts in vitro. J Cell Physiol. 1976 May;88(1):33–41. doi: 10.1002/jcp.1040880105. [DOI] [PubMed] [Google Scholar]
- Buchwald M. Abnormal levels of 3':5'-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis. Proc Natl Acad Sci U S A. 1976 Aug;73(8):2899–2903. doi: 10.1073/pnas.73.8.2899. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Corsaro C. M., Migeon B. R. Effect of ouabain resistance on human diploid fibroblasts carrying other genetic variants. Exp Cell Res. 1975 Oct 1;95(1):47–53. doi: 10.1016/0014-4827(75)90607-2. [DOI] [PubMed] [Google Scholar]
- DI SANT'AGNESE P. A., DARLING R. C., PERERA G. A., SHEA E. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease. Pediatrics. 1953 Nov;12(5):549–563. [PubMed] [Google Scholar]
- Lever J. E., Seegmiller J. E. Ouabain-resistant human lymphoblastoid lines altered in the (Na+ + K+)-dependent ATPase membrane transport system. J Cell Physiol. 1976 Jul;88(3):343–352. doi: 10.1002/jcp.1040880310. [DOI] [PubMed] [Google Scholar]
- Mangos J. A., McSherry N. R., Benke P. J. A sodium transport inhibitory factor in the saliva of patients with cystic fibrosis of the pancreas. Pediatr Res. 1967 Nov;1(6):436–442. doi: 10.1203/00006450-196711000-00002. [DOI] [PubMed] [Google Scholar]
- Mankovitz R., Buchwald M., Baker R. M. Isolation of ouabain-resistant human diploid fibroblasts. Cell. 1974 Nov;3(3):221–226. doi: 10.1016/0092-8674(74)90135-4. [DOI] [PubMed] [Google Scholar]
- Quissell D. O., Pitot H. C. Number of ouabain-binding sites in fibroblasts from normal subjects and patients with cystic fibrosis. Nature. 1974 Jan 11;247(5436):115–116. doi: 10.1038/247115a0. [DOI] [PubMed] [Google Scholar]
- SCHATZMANN H. J. Herzglykoside als Hemmstoffe für den aktiven Kalium- und Natriumtransport durch die Erythrocytenmembran. Helv Physiol Pharmacol Acta. 1953;11(4):346–354. [PubMed] [Google Scholar]
- Shank B. B., Smith N. E. Regulation of cellular growth by sodium pump activity. J Cell Physiol. 1976 Mar;87(3):377–387. doi: 10.1002/jcp.1040870313. [DOI] [PubMed] [Google Scholar]
- Taussig L. M., Gardner J. D. Effects of saliva and plasma from cystic fibrosis patients on membrane transport. Lancet. 1972 Jun 24;1(7765):1367–1368. doi: 10.1016/s0140-6736(72)91096-3. [DOI] [PubMed] [Google Scholar]