TY - GEN AU - Luciani,Alessandro AU - Villella,Valeria Rachela AU - Esposito,Speranza AU - Brunetti-Pierri,Nicola AU - Medina,Diego AU - Settembre,Carmine AU - Gavina,Manuela AU - Pulze,Laura AU - Giardino,Ida AU - Pettoello-Mantovani,Massimo AU - D'Apolito,Maria AU - Guido,Stefano AU - Masliah,Eliezer AU - Spencer,Brian AU - Quaratino,Sonia AU - Raia,Valeria AU - Ballabio,Andrea AU - Maiuri,Luigi TI - Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition SN - 1476-4679 PY - 2010///1022 KW - Acetylcysteine KW - pharmacology KW - Adaptor Proteins, Signal Transducing KW - genetics KW - Adolescent KW - Adult KW - Animals KW - Antioxidants KW - Apoptosis Regulatory Proteins KW - Autophagy KW - drug effects KW - Beclin-1 KW - Cell Line KW - Cystamine KW - Cystic Fibrosis KW - drug therapy KW - Cystic Fibrosis Transmembrane Conductance Regulator KW - antagonists & inhibitors KW - Epithelial Sodium Channels KW - GTP-Binding Proteins KW - Heat-Shock Proteins KW - metabolism KW - Humans KW - Inflammation KW - Membrane Proteins KW - Mice KW - Mice, Inbred CFTR KW - Mice, Inbred Strains KW - Mice, Transgenic KW - Microtubule-Associated Proteins KW - Models, Biological KW - Nasal Polyps KW - Organometallic Compounds KW - Phosphatidylinositol 3-Kinases KW - Phosphoinositide-3 Kinase Inhibitors KW - Protein Binding KW - physiology KW - Protein Glutamine gamma Glutamyltransferase 2 KW - Protein Transport KW - Reactive Oxygen Species KW - Respiratory Mucosa KW - cytology KW - Salicylates KW - Sequestosome-1 Protein KW - Small Ubiquitin-Related Modifier Proteins KW - Transglutaminases KW - Young Adult N1 - Publication Type: Journal Article; Research Support, Non-U.S. Gov't UR - https://doi.org/10.1038/ncb2090 ER -