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Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue

  • Researchers

    Nguyen Thu Ngan Trinh, Olivier Bardou, Anik Prive, Emilie Maille, Damien Adam, Sarah Lingee, Pasquale Ferraro, Martin-Yvon Desrosiers, Christelle Coraux, Emmanuelle Brochiero

  • Place of research

    University of Montreal, Canada

  • Publication

    European Respiratory Journal, December 2012

  • Subjects

    , ,

  • Do CFTR correctors have a beneficial effect on repair of CF lung tissue after injury?

  • Why is this important?

    CTFR – cystic fibrosis transmembrane conductance regulator - is a protein involved in the transfer of salt across the membranes of certain cells (epithelia). When the gene responsible for making these proteins mutates or changes from the norm it usually causes CF.

    In people with CF, chronic inflammation and infection causes damage to the cells (epithelia) that line the airways. This damage is a key component of the progression of CF lung disease, since it may further impair the defence against infection. Several years ago, we thus decided to devote our efforts to better understand the mechanisms controlling repair of these cells (epithelia) and identify strategies promoting airway regeneration.

  • What did you do?

    We first analysed the damage of the lung tissues in CF patients. We then grew cells in the lab from the lungs of people with and people without CF. We purposefully injured the cells and compared their rates of healing. The role of the CFTR protein in healing processes was also explored and a beneficial effect of CFTR correction was tested.

  • What did you find?

    We found that cells from the lungs of people with CF took longer to heal than cells from the lungs of people without CF. In fact, our results indicated that the basic CFTR defect could be responsible, at least in part, for the observed repair delay, even in absence of infection. Interestingly, we then demonstrated that growing the lung cells along with a CFTR corrector improved the wound-healing rates.

  • What does this mean and reasons for caution?

    Altogether, these data demonstrated that functional CFTR proteins play a critical role in airway cell repair, and treatment with CFTR correctors may represents a novel avenue to promote CF airway regeneration.

  • What's next?

    The next step will be to further study the mechanisms whereby CFTR controls the cell repair processes. Our goal will be to identify the most effective CFTR correctors in improving cell repair in infection-free conditions, as well as in the presence of infection.