Azithromycin Attenuates -Induced Lung Inflammation by Targeting Bacterial Proteins Secreted in the Cultured Medium.

Background: airway infections are a major cause of morbidity and mortality in patients with cystic fibrosis (CF). Azithromycin improves the related clinical outcomes, but its mechanisms of action remain poorly understood. We tested the hypothesis that azithromycin downregulates -induced pro-inflammatory responses by modifying release of bacterial proteins.

Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives.

Mutations of the gene cause cystic fibrosis (CF), the most common recessive monogenic disease worldwide. These mutations alter the synthesis, processing, function, or half-life of CFTR, the main chloride channel expressed in the apical membrane of epithelial cells in the airway, intestine, pancreas, and reproductive tract. Lung disease is the most critical manifestation of CF.

Strategies in early clinical development for the treatment of basic defects of cystic fibrosis.

Introduction: Twenty-six years after the identification of the gene responsible for cystic fibrosis (CF), controversies still surround the pathogenesis of the disease that continues to burden and shorten lives. Therefore, finding effective therapeutic strategies that target the basic defect of CF is crucially needed.

Areas Covered: This review offers a comprehensive survey of fundamental therapies in early stages of development for the treatment of CF.