Inflammatory stimuli and the rescue of disease-causing CFTR nonsense variants

<title>Extract</title> In the past decade or so, treatment of cystic fibrosis (CF) has changed enormously due to the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. While first covering a small percentage of individuals bearing specific disease-causing variants that affect gating and conductance of CFTR [1], the use of these types of therapy was later expanded with the approval of a combination including a corrector (that rescues folding and thus endoplasmic reticulum retention) and a potentiator (that rescues channel gating and conductance) that target the most common variant, F508del [2].