Emerging evidence suggests that several virulence factors and proteases released from Pseudomonas aeruginosa and inflammatory cells in CF airways have profound adverse effects on CFTR & ENaC function and may thus impede benefits of CFTR modulator therapies. On the other hand, restoration of mutant CFTR function may improve airway defenses and reduce inflammation and infection in CF. This symposium will review and integrate the latest data in this field, allowing the participant to better understand the interrelation between CF ion transport defects, infection & inflammation and potential implications for CFTR modulator therapies as well as strategies targeting alternative targets such as ENaC
Describe unwanted effects of proteases released in CF airways' infection & inflammation on CFTR & ENaC and discuss consequences for CF ion transport defects.
Describe impact of Pseudomonas aeruginosa infection on wild-type CFTR function and on pharmacological rescue of F508del-CFTR.
Summarize latest data on effects of CFTR modulator therapy on infection & inflammation in CF airways.