To determine a potential role for CFTR dysfunction in the development of emphysema, Cftr<sup>+/+</sup> (Wild-type; WT), Cftr<sup>+/-</sup> (heterozygous), and Cftr<sup>-/-</sup> (knock-out; KO) mice were aged or exposed to cigarette smoke and analyzed for airspace enlargement.
In addition, proposed nano-based autophagy induction strategy can also allow rescue of cigarette smoke (CS) induced acquired-CFTR dysfunction seen in chronic obstructive pulmonary disease (COPD)-emphysema subjects.
Our results indicate that inhibition of de novo ceramide synthesis may be effective in disease states with low CFTR expression like emphysema and chronic lung injury but not in complete absence of lipid-raft CFTR as in ΔF508-cystic fibrosis.
These results confirm the involvement of the CFTR gene in disseminated bronchiectasis of unknown origin, and suggest a possible role for CFTR gene mutations in sarcoidosis, and for the 5T allele in pulmonary emphysema.