Background Mucus stasis in chronic obstructive pulmonary disease (COPD) is a significant
contributor to morbidity and mortality. Potentiators of cystic fibrosis transmembrane …

We demonstrate the use of a high resolution form of optical coherence tomography, termed
micro-OCT (μOCT), for investigating the functional microanatomy of airway epithelia. μOCT …

The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-dependent protein
kinase A-activated chloride channel that resides on the apical surface of epithelial cells. …

Rationale: The mechanisms underlying cystic fibrosis (CF) lung disease pathogenesis are
unknown. Objectives: To establish mechanisms linking anion transport with the functional …

Environmental insults and misfolded proteins cause endoplasmic reticulum (ER) stress and
activate the unfolded protein response (UPR). The UPR decreases endogenous cystic …

Rationale: Premature termination codons (PTCs) in the cystic fibrosis transmembrane
conductance regulator (CFTR) gene cause cystic fibrosis (CF). Several agents are known to …

Cigarette smoking causes acquired cystic fibrosis transmembrane conductance regulator (CFTR)
dysfunction and is associated with delayed mucociliary clearance and chronic …

Acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction may
contribute to chronic obstructive pulmonary disease pathogenesis and is a potential therapeutic …

Certain aminoglycosides are capable of inducing “translational readthrough” of premature
termination codons (PTCs). However, toxicity and relative lack of efficacy deter treatment with …

Cigarette smoking is associated with chronic obstructive pulmonary disease and chronic
bronchitis. Acquired ion transport abnormalities, including cystic fibrosis transmembrane …