Role of Ca2+-activated ion transport in the treatment of cystic fibrosis

A. Zsembery, Dóra Hargitai

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Cystic fibrosis (CF) is caused by defective cyclic AMP-dependent cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel. Therefore, CF epithelial cells fail to transport, Cl- and water. Furthermore, the cessation of Cl- efflux across the apical membrane of CF pancreatic and biliary duct cells reduces HCO3 - secretion as well. In CF epithelial cells activation of calcium-dependent Cl- channels might substitute for impaired CFTR function and restore Cl- and/or HCO3 - secretion. ATP-mediated stimulation of P2Y and P2X purinergic receptors causes an increase in cytosolic Ca2+ concentration ([Ca2+]i). Effects of ATP are influenced by external zinc, pH and Na+ concentrations. In low Na+, alkaline environment, ATP and zinc induce a sustained and reproducible Ca2+ signal because of P2X receptor mediated Ca 2+ influx from the extracellular space. Importantly, the increase in [Ca2+]i stimulates anion secretion of nasal epithelia in CF mouse models suggesting that targeting P2X receptors might have beneficial effects in CF therapy.

Original languageEnglish
Pages (from-to)562-564
Number of pages3
JournalWiener Medizinische Wochenschrift
Volume158
Issue number19-20
DOIs
Publication statusPublished - Oct 2008

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Ion Transport
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Adenosine Triphosphate
Zinc
Purinergic P2Y Receptors
Purinergic P2X Receptors
Epithelial Cells
Nasal Mucosa
Pancreatic Ducts
Extracellular Space
Cyclic AMP
Anions
Calcium
Membranes
Water

Keywords

  • Bicarbonate
  • CFTR
  • Intracellular pH
  • Purinergic signaling
  • Zinc

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Role of Ca2+-activated ion transport in the treatment of cystic fibrosis. / Zsembery, A.; Hargitai, Dóra.

In: Wiener Medizinische Wochenschrift, Vol. 158, No. 19-20, 10.2008, p. 562-564.

Research output: Contribution to journalArticle

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