Inflammatory response in CF airway epithelial cells: a comparative study of modulators and wild-type CFTR rescue.

ABSTRACT: The combination of pharmacological modulators such as lumacaftor, tezacaftor, and elexacaftor restore CFTR activity at the plasma membrane and improve lung function in patients carrying CFTR mutations such as F508del, their effects on inflammation are less clear. This study aimed to investigate whether the inflammatory response in CF airway epithelial cells depends solely on Cl^- transport or also on the structural integrity of the CFTR protein. We examined the effects of several CFTR modulators and wild-type CFTR overexpression on CFTR expression, trafficking, Cl^- channel activity, and inflammation in human CF bronchial epithelial cells. Our results demonstrate that overexpression of wild-type CFTR fully restores Cl^- secretion and normalizes the inflammator

SUBMITTER: Kouadri A 

PROVIDER: S-EPMC12756383 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature