CFTR and cystic fibrosis: There is also an absence of PERK-eIF2α activity in the CF lung and in cultured AECs from ΔF508-CFTR patients, but activation of this pathway using an eIF2α agonist beneficially attenuated the robust inflammatory response to flagellin and P. aeruginosa (Nanua et al., 2006; Blohmke et al., 2012).