Transport properties in CFTR−/− knockout piglets suggest normal airway surface liquid pH and enhanced amiloride-sensitive Na+ absorption

  1. Benedetto, Roberta
  2. Centeio, Raquel
  3. Ousingsawat, Jiraporn
  4. Schreiber, Rainer
  5. Janda, Melanie
  6. Kunzelmann, Karl ORCID logo

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1000 Titel
  • Transport properties in CFTR−/− knockout piglets suggest normal airway surface liquid pH and enhanced amiloride-sensitive Na+ absorption
1000 Autor/in
  1. Benedetto, Roberta |
  2. Centeio, Raquel |
  3. Ousingsawat, Jiraporn |
  4. Schreiber, Rainer |
  5. Janda, Melanie |
  6. Kunzelmann, Karl |
1000 Erscheinungsjahr 2020
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2020-07-25
1000 Erschienen in
1000 Quellenangabe
  • 472(10):1507-1519
1000 Copyrightjahr
  • 2020
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1007/s00424-020-02440-y |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7476968/ |
1000 Publikationsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • Previous analysis of CFTR-knockout (CFTR-/-) in piglets has provided important insights into the pathology of cystic fibrosis. However, controversies exist as to the true contribution of CFTR to the pH balance in airways and intestine. We therefore compared ion transport properties in newborn wild-type (CFTR+/+) and CFTR-knockout (CFTR-/- piglets). Tracheas of CFTR-/- piglets demonstrated typical cartilage malformations and muscle cell bundles. CFTR-/- airway epithelial cells showed enhanced lipid peroxidation, suggesting inflammation early in life. CFTR was mainly expressed in airway submucosal glands and was absent in lungs of CFTR-/- piglets, while expression of TMEM16A was uncompromised. mRNA levels for TMEM16A, TMEM16F, and αβγENaC were unchanged in CFTR-/- airways, while mRNA for SLC26A9 appeared reduced. CFTR was undetectable in epithelial cells of CFTR-/- airways and intestine. Small intestinal epithelium of CFTR-/- piglets showed mucus accumulation. Secretion of both electrolytes and mucus was activated by stimulation with prostaglandin E2 and ATP in the intestine of CFTR+/+, but not of CFTR-/- animals. pH was measured inside small bronchi using a pH microelectrode and revealed no difference between CFTR+/+ and CFTR-/- piglets. Intracellular pH in porcine airway epithelial cells revealed only a small contribution of CFTR to bicarbonate secretion, which was absent in cells from CFTR-/- piglets. In contrast to earlier reports, our data suggest a minor impact of CFTR on ASL pH. In contrast, enhanced amiloride-sensitive Na
1000 Sacherschließung
lokal Epithelial Sodium Channel Blockers/pharmacology [MeSH]
lokal Bronchi/cytology [MeSH]
lokal Respiratory Tract Absorption [MeSH]
lokal Cystic Fibrosis Transmembrane Conductance Regulator/genetics [MeSH]
lokal Airways
lokal Cystic fibrosis
lokal Organ Physiology
lokal Respiratory Mucosa/drug effects [MeSH]
lokal Respiratory Mucosa/metabolism [MeSH]
lokal Cystic Fibrosis/metabolism [MeSH]
lokal Bicarbonate transport
lokal Sodium/metabolism [MeSH]
lokal Intestinal Mucosa/drug effects [MeSH]
lokal Anoctamins/metabolism [MeSH]
lokal Swine [MeSH]
lokal Amiloride/pharmacology [MeSH]
lokal Intestinal Absorption [MeSH]
lokal Animals [MeSH]
lokal Anoctamins/genetics [MeSH]
lokal Chloride secretion
lokal Bronchi/metabolism [MeSH]
lokal Intestinal Mucosa/metabolism [MeSH]
lokal CFTR−/− piglets
lokal CFTR
lokal Hydrogen-Ion Concentration [MeSH]
lokal Intestinal epithelium
lokal Epithelial Sodium Channels/metabolism [MeSH]
lokal Cells, Cultured [MeSH]
lokal Epithelial Sodium Channels/genetics [MeSH]
1000 Liste der Beteiligten
  1. https://frl.publisso.de/adhoc/uri/QmVuZWRldHRvLCBSb2JlcnRh|https://frl.publisso.de/adhoc/uri/Q2VudGVpbywgUmFxdWVs|https://frl.publisso.de/adhoc/uri/T3VzaW5nc2F3YXQsIEppcmFwb3Ju|https://frl.publisso.de/adhoc/uri/U2NocmVpYmVyLCBSYWluZXI=|https://frl.publisso.de/adhoc/uri/SmFuZGEsIE1lbGFuaWU=|https://orcid.org/0000-0001-6222-4593
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1000 Erstellt am 2023-11-17T09:05:21.850+0100
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