Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition. [electronic resource]
Producer: 20101022Description: 863-75 p. digitalISSN:- 1476-4679
- 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
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Publication Type: Journal Article; Research Support, Non-U.S. Gov't
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