Cystic Fibrosis (CF) is a genetic disease that affects the respiratory and digestive system and is most common among Caucasians of Northern European origin. CF is caused by mutations in a membrane protein CFTR (Cystic Fibrosis Transmembrane-conductance Regulator). This mutation impairs the membrane protein’s chloride ion channel function. One of the most common CFTR mutations is the DF508 mutation that affects over 70% of CF cases. Our research has shown that the DF508-CFTR mutation can be partially reversed by physical and chemical means [Heda & Marino, BBRC, 271:659-664, 2000]. In cell lines expressing DF508-CFTR, synthetic anion carriers have shown to augment the chloride ion channel function (Yang et al. J. Biol. Chem.,1278(37) : 35079-35085, 2003). In this study, we use triazole compounds, synthesized in Talukdar lab (IISER, Pune, India), to determine their effects on DF508-CFTR upregulation. These compounds are known for their ability to bind and facilitate chloride influx to cultured cell lines. Methods: Human lung epithelial cell lines (CFBE) transfected with DF508-CFTR were cultured and treated with various concentrations of triazole compounds (ABS-089, PJ-08) at 27 degrees for 60 hours. Cell lysates were then prepared and immunoblotted with anti-CFTR antibody and CFTR-specific signal was detected by chemiluminescence using c300 image analyzer (Azure Biosystems). Results: Both tested compounds showed an increase in the CFTR band-B when compared to the vehicle alone. Conclusion: Our research suggests triazole compounds could potentially be used as therapeutic agents for Cystic Fibrosis treatment
