Home LiteratureArticle Details
PMID: 3771796 Published · ppublish English Comparative Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation.

The Journal of clinical investigation ·Vol. 78 ·No. 5 ·1986-11-00 ·Pages 1245-52

Boucher RC, Stutts MJ, Knowles MR, Cantley L, Gatzy JT

Abstract

The transepithelial potential difference (PD) of cystic fibrosis (CF) airway epithelium is abnormally raised and the Cl- permeability is low. We studied the contribution of active Na+ absorption to the PD and attempted to increase the Cl- permeability of CF epithelia. Nasal epithelia from CF and control subjects were mounted in Ussing chambers and were short-circuited. The basal rate of Na+ absorption was raised in CF polyps compared with control tissues. Whereas beta agonists induced Cl- secretion in normal and atopic epithelia, beta agonists further increased the rate of Na+ absorption in CF epithelia without inducing Cl- secretion. This unusual effect is not due to an abnormal CF beta receptor because similar effects were induced by forskolin, and because cAMP production was similar in normal and CF epithelia. We conclude that CF airway epithelia absorb Na+ at an accelerated rate. The abnormal response to beta agonists may reflect a primary abnormality in a cAMP-modulated path, or a normal cAMP-modulated process in a Cl- impermeable epithelial cell.

MeSH Terms
Adenylyl Cyclases/metabolism Adolescent Adult Child Cystic Fibrosis/metabolism,physiopathology Enzyme Activation Epithelium/drug effects,metabolism,physiology Female Humans In Vitro Techniques Indomethacin/pharmacology Isoproterenol/pharmacology Kinetics Male Nasal Mucosa/metabolism Reference Values Sodium/metabolism
Chemicals
Sodium Adenylyl Cyclases Isoproterenol Indomethacin
Authors & Affiliations
5 authors, click to expand affiliations / ORCID
Boucher R C
Stutts M J
Knowles M R
Cantley L
Gatzy J T
References (28)
28 references, click to expand
  1. Studies on pulmonary secretions. II. Osmolality and the ionic environment of pulmonary secretions from patients with cystic fibrosis, bronchiectasis, and laryngectomy.
    Am Rev Respir Dis. 1967 Jul;96(1):83-7 PMID: 6027728
  2. Composition of tracheobronchial secretions in cystic fibrosis of the pancreas and bronchiectasis.
    Pediatrics. 1959 Nov;24:739-45 PMID: 13809672
  3. Active transport of Na+ and Cl- across the canine tracheal epithelium in vitro.
    Am Rev Respir Dis. 1975 Dec;112(6):811-5 PMID: 1202998
  4. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding.
    Anal Biochem. 1976 May 7;72:248-54 PMID: 942051
  5. Na and Cl transport across the isolated turtle colon: parallel pathways for transmural ion movement.
    J Membr Biol. 1977 Dec 15;37(3-4):213-33 PMID: 599551
  6. Decreased adenosine 3':5'-monophosphate response to isoproterenol in cystic fibrosis leukocytes.
    Pediatr Res. 1978 Jun;12(6):703-7 PMID: 209391
  7. Effect of catecholamines on ion transport in dog tracheal epithelium.
    J Appl Physiol Respir Environ Exerc Physiol. 1979 Aug;47(2):397-403 PMID: 468698
  8. Abnormal adrenergic and cholinergic sensitivity in cystic fibrosis.
    N Engl J Med. 1980 Jun 26;302(26):1453-6 PMID: 6246430
  9. Pathways of ion movement in the canine tracheal epithelium.
    Am J Physiol. 1980 Sep;239(3):F215-21 PMID: 7435560
  10. Current-voltage analysis of apical sodium transport in toad urinary bladder: effects of inhibitors of transport and metabolism.
    J Membr Biol. 1980 Nov 15;57(1):59-71 PMID: 6256553
  11. Modification of Na and Cl transport in canine tracheal mucosa by prostaglandins.
    Am J Physiol. 1981 Feb;240(2):F101-5 PMID: 6258446
  12. Impaired beta adrenergic receptor binding and function in cystic fibrosis neutrophils.
    J Clin Invest. 1981 Jul;68(1):253-8 PMID: 6265498
  13. Forskolin: unique diterpene activator of adenylate cyclase in membranes and in intact cells.
    Proc Natl Acad Sci U S A. 1981 Jun;78(6):3363-7 PMID: 6267587
  14. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis.
    N Engl J Med. 1981 Dec 17;305(25):1489-95 PMID: 7300874
  15. Hormonal control of chloride secretion by canine tracheal epithelium: an electrophysiologic analysis.
    Ann N Y Acad Sci. 1981;372:558-70 PMID: 6280555
  16. Neurohormonal receptors and cyclic AMP-binding proteins in rabbit tracheal mucosa-submucosa.
    Biochim Biophys Acta. 1982 Nov 24;719(2):169-77 PMID: 6129899
  17. Chloride impermeability in cystic fibrosis.
    Nature. 1983 Feb 3;301(5899):421-2 PMID: 6823316
  18. 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):1185-9 PMID: 6843595
  19. Intracellular chloride activities in canine tracheal epithelium. Direct evidence for sodium-coupled intracellular chloride accumulation in a chloride-secreting epithelium.
    J Clin Invest. 1983 May;71(5):1392-401 PMID: 6853719
  20. Relative ion permeability of normal and cystic fibrosis nasal epithelium.
    J Clin Invest. 1983 May;71(5):1410-7 PMID: 6853720
  21. Beta adrenergic receptors in lymphocytes and granulocytes from patients with cystic fibrosis.
    J Clin Invest. 1983 Jun;71(6):1787-95 PMID: 6306054
  22. Abnormal ion permeation through cystic fibrosis respiratory epithelium.
    Science. 1983 Sep 9;221(4615):1067-70 PMID: 6308769
  23. Chloride secretion by canine tracheal epithelium: I. Role of intracellular c AMP levels.
    J Membr Biol. 1982;70(3):217-26 PMID: 6313924
  24. Chloride secretion by canine tracheal epithelium: II. The cellular electrical potential profile.
    J Membr Biol. 1982;70(3):227-38 PMID: 6313925
  25. Bioelectric properties and ion flow across excised human bronchi.
    J Appl Physiol Respir Environ Exerc Physiol. 1984 Apr;56(4):868-77 PMID: 6144659
  26. Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro.
    J Clin Invest. 1984 Jun;73(6):1763-71 PMID: 6327771
  27. Determination of the electromotive force of active sodium transport in frog skin epithelium (Rana temporaria) from presteady-state flux ratio experiments.
    J Membr Biol. 1985;86(2):105-11 PMID: 3875727
  28. Radioimmunoassay for cyclic nucleotides. I. Preparation of antibodies and iodinated cyclic nucleotides.
    J Biol Chem. 1972 Feb 25;247(4):1106-13 PMID: 4334491
Article Info
Journal
The Journal of clinical investigation
Abbr.
J Clin Invest
ISSN
0021-9738
DOI
10.1172/JCI112708
Published
1986-11-00
Pages
1245-52
Language
English
Region
United States
NLM ID
7802877
PMCID
PMC423810
Subset
IM
Grants
NHLBI NIH HHS · HL00787 · United States
NHLBI NIH HHS · HL22924 · United States
External Links
PubMed source DOI: 10.1172/JCI112708 PMC Full Text View journal details
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: [email protected]

Copyright © 2026.Genelibs Jinan Honghui Biotechnology Co., Ltd. 鲁ICP备20007159号-2