CFTR and cystic fibrosis: Notably, a specific analogue of adamantanemethyloxypentyl-1-deoxynojirimycin (AMP-DNM), a potent inhibitor of the non-lysosomal β-glucosidase 2 enzyme, involved in sphingolipid metabolism and P. aeruginosa-induced inflammation [133], proved to effectively correct F508del-CFTR in CF-KM4 cells [134].