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The Role Of Cystic Fibrosis Conductance Regulator In The Clearance Of Burkholderia Cenocepacia By Macrophages, Hanna Ostapska

Electronic Thesis and Dissertation Repository

Burkholderia cenocepacia is an opportunistic Gram-negative bacterium that causes chronic respiratory infection in cystic fibrosis (CF) patients, leading to rapid decline of lung function. CF patients harbour mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride (Cl) transport channel. B. cenocepacia survive in macrophages by delaying phagosome maturation and exaggerate this strategy in CFTR-defective macrophages. Neutrophils of CF patients display defective Cl transport resulting in impaired chlorination of bacteria via hypochlorous acid (HOCl), resulting in reduced bacterial clearance. I hypothesize that macrophages with dysfunctional CFTR lack sufficient Cl ion levels in their phagosomes for the …