Chronic inflammation in CF airways - a persistent issue for A20

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    Cystic Fibrosis (CF) is characterised by prolonged and exaggerated airways inflammation. Despite recent developments to overcome the underlying functional defect in CFTR (cystic fibrosis transmembrane conductance regulator), there is still an unmet need to reduce the inflammatory response. The NF-kB regulator A20 is a key target to normalise the inflammatory response and is reduced in CF. Here, we describe the plethora of functions of A20 as they apply to innate immune function within the airways. Pharmacological compounds can enhance A20 mRNA and protein expression, but we observed a blunted effect in CF primary epithelial cells. In CF cells pre-treatment with gibberellic acid (GA3) shows anti-inflammatory effects only in some patients. We show that cells with higher basal p38 expression respond with an increase in pro-inflammatory cytokines. Furthermore, all CF PNECs show increased p38 mRNA when stimulated in the presence of GA3. Our results suggest that those patients may benefit from therapeutics targeting p38.


    • chronic-inflammation-in-cf-airways--a-persistent-issue-for-a20-2157-7412-1000304

      Rights statement: © 2016 The Authors. This is an open access article published under a Creative Commons Attribution License (, which permits unrestricted use, distribution and reproduction in any medium, javascript:void(0);provided the author and source are cited.

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    Original languageEnglish
    JournalJournal of Genetic Syndromes & Gene Therapy
    Journal publication date19 Aug 2016
    Publication statusPublished - 19 Aug 2016

    ID: 94993303