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. 1987 Dec;80(6):1523–1526. doi: 10.1172/JCI113237

Cystic fibrosis.

M J Welsh 1, R B Fick 1
PMCID: PMC442419  PMID: 3316277

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bijman J., Quinton P. Permeability properties of cell membranes and tight junctions of normal and cystic fibrosis sweat ducts. Pflugers Arch. 1987 May;408(5):505–510. doi: 10.1007/BF00585076. [DOI] [PubMed] [Google Scholar]
  2. Boucher R. C., Stutts M. J., Knowles M. R., Cantley L., Gatzy J. T. Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J Clin Invest. 1986 Nov;78(5):1245–1252. doi: 10.1172/JCI112708. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Campbell D. J. Circulating and tissue angiotensin systems. J Clin Invest. 1987 Jan;79(1):1–6. doi: 10.1172/JCI112768. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Cotton C. U., Stutts M. J., Knowles M. R., Gatzy J. T., Boucher R. C. Abnormal apical cell membrane in cystic fibrosis respiratory epithelium. An in vitro electrophysiologic analysis. J Clin Invest. 1987 Jan;79(1):80–85. doi: 10.1172/JCI112812. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Dorin J. R., Novak M., Hill R. E., Brock D. J., Secher D. S., van Heyningen V. A clue to the basic defect in cystic fibrosis from cloning the CF antigen gene. Nature. 1987 Apr 9;326(6113):614–617. doi: 10.1038/326614a0. [DOI] [PubMed] [Google Scholar]
  6. Frizzell R. A., Rechkemmer G., Shoemaker R. L. Altered regulation of airway epithelial cell chloride channels in cystic fibrosis. Science. 1986 Aug 1;233(4763):558–560. doi: 10.1126/science.2425436. [DOI] [PubMed] [Google Scholar]
  7. Goodfellow P. N. Cystic fibrosis. Classical and reverse genetics. 1987 Apr 30-May 6Nature. 326(6116):824–824. doi: 10.1038/326824a0. [DOI] [PubMed] [Google Scholar]
  8. Hamill O. P., Marty A., Neher E., Sakmann B., Sigworth F. J. Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches. Pflugers Arch. 1981 Aug;391(2):85–100. doi: 10.1007/BF00656997. [DOI] [PubMed] [Google Scholar]
  9. Knowles M. R., Stutts M. J., Spock A., Fischer N., Gatzy J. T., Boucher R. C. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science. 1983 Sep 9;221(4615):1067–1070. doi: 10.1126/science.6308769. [DOI] [PubMed] [Google Scholar]
  10. Knowles M., Gatzy J., Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med. 1981 Dec 17;305(25):1489–1495. doi: 10.1056/NEJM198112173052502. [DOI] [PubMed] [Google Scholar]
  11. Knowles M., Gatzy J., Boucher R. Relative ion permeability of normal and cystic fibrosis nasal epithelium. J Clin Invest. 1983 May;71(5):1410–1417. doi: 10.1172/JCI110894. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Kopelman H., Durie P., Gaskin K., Weizman Z., Forstner G. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med. 1985 Feb 7;312(6):329–334. doi: 10.1056/NEJM198502073120601. [DOI] [PubMed] [Google Scholar]
  13. Manson J. C., Brock D. J. Development of a quantitative immunoassay for the cystic fibrosis gene. Lancet. 1980 Feb 16;1(8164):330–331. doi: 10.1016/s0140-6736(80)90882-x. [DOI] [PubMed] [Google Scholar]
  14. Margolies R., Boat T. F. The carbohydrate content of IgG from patients with cystic fibrosis. Pediatr Res. 1983 Dec;17(12):931–935. doi: 10.1203/00006450-198312000-00001. [DOI] [PubMed] [Google Scholar]
  15. McPherson M. A., Dormer R. L., Dodge J. A., Goodchild M. C. Adrenergic secretory responses of submandibular tissues from control subjects and cystic fibrosis patients. Clin Chim Acta. 1985 Jun 14;148(3):229–237. doi: 10.1016/0009-8981(85)90149-4. [DOI] [PubMed] [Google Scholar]
  16. Newmark P. Testing for cystic fibrosis. 1985 Nov 28-Dec 4Nature. 318(6044):309–309. doi: 10.1038/318309a0. [DOI] [PubMed] [Google Scholar]
  17. Quinton P. M., Bijman J. Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis. N Engl J Med. 1983 May 19;308(20):1185–1189. doi: 10.1056/NEJM198305193082002. [DOI] [PubMed] [Google Scholar]
  18. Quinton P. M. Chloride impermeability in cystic fibrosis. Nature. 1983 Feb 3;301(5899):421–422. doi: 10.1038/301421a0. [DOI] [PubMed] [Google Scholar]
  19. Quinton P. M. Missing Cl conductance in cystic fibrosis. Am J Physiol. 1986 Oct;251(4 Pt 1):C649–C652. doi: 10.1152/ajpcell.1986.251.4.C649. [DOI] [PubMed] [Google Scholar]
  20. Reitman M. L., Varki A., Kornfeld S. Fibroblasts from patients with I-cell disease and pseudo-Hurler polydystrophy are deficient in uridine 5'-diphosphate-N-acetylglucosamine: glycoprotein N-acetylglucosaminylphosphotransferase activity. J Clin Invest. 1981 May;67(5):1574–1579. doi: 10.1172/JCI110189. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Sato K., Sato F. Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro. J Clin Invest. 1984 Jun;73(6):1763–1771. doi: 10.1172/JCI111385. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Scanlin T. F., Wang Y. M., Glick M. C. Altered fucosylation of membrane glycoproteins from cystic fibrosis fibroblasts. Pediatr Res. 1985 Apr;19(4):368–374. doi: 10.1203/00006450-198519040-00011. [DOI] [PubMed] [Google Scholar]
  23. Welsh M. J. An apical-membrane chloride channel in human tracheal epithelium. Science. 1986 Jun 27;232(4758):1648–1650. doi: 10.1126/science.2424085. [DOI] [PubMed] [Google Scholar]
  24. Welsh M. J. Electrolyte transport by airway epithelia. Physiol Rev. 1987 Oct;67(4):1143–1184. doi: 10.1152/physrev.1987.67.4.1143. [DOI] [PubMed] [Google Scholar]
  25. Welsh M. J., Liedtke C. M. Chloride and potassium channels in cystic fibrosis airway epithelia. 1986 Jul 31-Aug 6Nature. 322(6078):467–470. doi: 10.1038/322467a0. [DOI] [PubMed] [Google Scholar]
  26. Widdicombe J. H. Cystic fibrosis and beta-adrenergic response of airway epithelial cell cultures. Am J Physiol. 1986 Oct;251(4 Pt 2):R818–R822. doi: 10.1152/ajpregu.1986.251.4.R818. [DOI] [PubMed] [Google Scholar]
  27. Widdicombe J. H., Welsh M. J., Finkbeiner W. E. Cystic fibrosis decreases the apical membrane chloride permeability of monolayers cultured from cells of tracheal epithelium. Proc Natl Acad Sci U S A. 1985 Sep;82(18):6167–6171. doi: 10.1073/pnas.82.18.6167. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Wilson G. B., Jahn T. L., Fonseca J. R. Demonstration of serum protein differences in cystic fibrosis by isoelectric focusing in thin-layer polyacrylamide gels. Clin Chim Acta. 1973 Nov 23;49(1):79–91. doi: 10.1016/0009-8981(73)90346-x. [DOI] [PubMed] [Google Scholar]
  29. Yankaskas J. R., Cotton C. U., Knowles M. R., Gatzy J. T., Boucher R. C. Culture of human nasal epithelial cells on collagen matrix supports. A comparison of bioelectric properties of normal and cystic fibrosis epithelia. Am Rev Respir Dis. 1985 Dec;132(6):1281–1287. doi: 10.1164/arrd.1985.132.6.1281. [DOI] [PubMed] [Google Scholar]
  30. Yankaskas J. R., Knowles M. R., Gatzy J. T., Boucher R. C. Persistence of abnormal chloride ion permeability in cystic fibrosis nasal epithelial cells in heterologous culture. Lancet. 1985 Apr 27;1(8435):954–956. doi: 10.1016/s0140-6736(85)91728-3. [DOI] [PubMed] [Google Scholar]
  31. van Heyningen V., Hayward C., Fletcher J., McAuley C. Tissue localization and chromosomal assignment of a serum protein that tracks the cystic fibrosis gene. Nature. 1985 Jun 6;315(6019):513–515. doi: 10.1038/315513a0. [DOI] [PubMed] [Google Scholar]

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