Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells. (Articolo in rivista)

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Label
  • Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells. (Articolo in rivista) (literal)
Anno
  • 2012-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.3389/fphar.2012.00176 (literal)
Alternative label
  • Donglei Zhang1*+, Fabiana Ciciriello1*+, Suzana M. Anjos1, Annamaria Carissimo2, Jie Liao3, Graeme W. Carlile1, Haouaria Balghi3, Renaud Robert3, Alberto Luini2,4, John W. Hanrahan3 and David Y. Thomas1 (2012)
    Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells.
    in Symposia on frontiers of pharmacology
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Donglei Zhang1*+, Fabiana Ciciriello1*+, Suzana M. Anjos1, Annamaria Carissimo2, Jie Liao3, Graeme W. Carlile1, Haouaria Balghi3, Renaud Robert3, Alberto Luini2,4, John W. Hanrahan3 and David Y. Thomas1 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#url
  • http://www.frontiersin.org/Pharmacology_of_Ion_Channels_and_Channelopathies/10.3389/fphar.2012.00176/abstract (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 3 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroFascicolo
  • 176 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • 1Department of Biochemistry, McGill University, Montréal, QC, Canada 2Telethon Institute of Genetics and Medicine, Naples, Italy 3Department of Physiology, McGill University, Montréal, QC, Canada 4Institute of Protein Biochemistry, National Research Council, Naples, Italy (literal)
Titolo
  • Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells. (literal)
Abstract
  • Most cases of cystic fibrosis (CF) are caused by the deletion of a single phenylalanine residue at position 508 of the cystic fibrosis transmembrane conductance regulator (CFTR). The mutant F508del-CFTR is retained in the endoplasmic reticulum and degraded, but can be induced by low temperature incubation (29°C) to traffic to the plasma membrane where it functions as a chloride channel. Here we show that, cardiac glycosides, at nanomolar concentrations, can partially correct the trafficking of F508del-CFTR in human CF bronchial epithelial cells (CFBE41o-) and in an F508del-CFTR mouse model. Comparison of the transcriptional profiles obtained with polarized CFBE41o-cells after treatment with ouabain and by low temperature has revealed a striking similarity between the two corrector treatments that is not shared with other correctors. In summary, our study shows a novel function of ouabain and its analogs in the regulation of F508del-CFTR trafficking and suggests that compounds that mimic this low temperature correction of trafficking will provide new avenues for the development of therapeutics for CF. (literal)
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