What is it about?
QUB-TL1 is a novel compound, restricted to the extracellular surface of airway epithelial cells (AECs), that selectively inhibits critical channel activating proteases of the epithelial sodium channel (ENaC). QUB-TL1-mediated inhibition of ENaC in AECs obtained from cystic fibrosis patients (F508del homozygotes) improves airways hydration and restores mucociliary function.
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Why is it important?
Restoration of effective mucociliary clearance in CF could help keep the airways clear of infection. This study therefore provides evidence that protease inhibitor compounds such as QUB-TL1 have potential to significantly reduce the impact of CF lung disease.
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This page is a summary of: Inhibition of Protease–Epithelial Sodium Channel Signaling Improves Mucociliary Function in Cystic Fibrosis Airways, American Review of Respiratory Disease, September 2016, American Thoracic Society,
DOI: 10.1164/rccm.201511-2216oc.
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