Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition.

Luciani, Alessandro

Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition. [electronic resource] - Nature cell biology Sep 2010 - 863-75 p. digital

Publication Type: Journal Article; Research Support, Non-U.S. Gov't

1476-4679

10.1038/ncb2090 doi


Acetylcysteine--pharmacology
Adaptor Proteins, Signal Transducing--genetics
Adolescent
Adult
Animals
Antioxidants--pharmacology
Apoptosis Regulatory Proteins--genetics
Autophagy--drug effects
Beclin-1
Cell Line
Cystamine--pharmacology
Cystic Fibrosis--drug therapy
Cystic Fibrosis Transmembrane Conductance Regulator--antagonists & inhibitors
Epithelial Sodium Channels--genetics
GTP-Binding Proteins
Heat-Shock Proteins--metabolism
Humans
Inflammation--metabolism
Membrane Proteins--genetics
Mice
Mice, Inbred CFTR
Mice, Inbred Strains
Mice, Transgenic
Microtubule-Associated Proteins--metabolism
Models, Biological
Nasal Polyps--drug therapy
Organometallic Compounds--pharmacology
Phosphatidylinositol 3-Kinases--metabolism
Phosphoinositide-3 Kinase Inhibitors
Protein Binding--physiology
Protein Glutamine gamma Glutamyltransferase 2
Protein Transport--genetics
Reactive Oxygen Species--antagonists & inhibitors
Respiratory Mucosa--cytology
Salicylates--pharmacology
Sequestosome-1 Protein
Small Ubiquitin-Related Modifier Proteins--metabolism
Transglutaminases--genetics
Young Adult