QBW276

QBW276 is an investigational small molecule drug developed as an inhaled therapy for cystic fibrosis. It acts as a blocker of the epithelial sodium channel (ENaC), aiming to restore airway surface liquid hydration and improve mucociliary clearance in patients with cystic fibrosis. The drug was designed to be mutation-agnostic, potentially benefiting all individuals with CF regardless of their specific genetic mutations. Clinical trials showed that QBW276 is rapidly absorbed into the bloodstream after inhalation and quickly eliminated, with low systemic exposure and short half-lives for both the parent compound and its metabolites. Development of QBW276 for cystic fibrosis was discontinued before completion of late-stage clinical trials[1][2][5].