Researchers aim to evaluate the functionality of the CFTR channel after treatment with a new molecule (NXV) that they identified in a precedent FFC project (1/2014). NXV was identified by computational and biological screening of special libraries and is now under patent processing. Further, the activity of a new lead molecule(s) in cells stably expressing a nonsense-CFTR mRNA (ns CFTR) in CF cellular model systems will be investigated. They also will study the supramolecular interactions among TRIDs (Translational Read-Through-Inducing Drugs), CFTR mRNA and the ribosomal A-site to identify the biological target and the mechanism of action. FRT cells engineered with the vector expressing mutagenized ns CFTR, and nonsense-CF-human broncoepithelial cells grown in the air-liquid culture system to reproduce in vitro the epithelial organization will be used. CFTR expression after treatments with the new molecules will be evaluated by biomolecular techniques; CFTR activity will be revealed by specific CFTR functionality assays. Finally, in vitro-in vivo (Zebrafish model) analysis of the safety profile for the set of synthesized molecule will complete the study.