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Autophagy, ISSN 1554-8627, 11/2014, Volume 10, Issue 11, pp. 2053 - 2074
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as... 
PM, plasma membrane | autophagy | epigallocatechin gallate | TNF, tumor necrosis factor | CHX, cycloheximide | CXCL2, chemokine (C-X-C motif) ligand 2 | TGM2, transglutaminase 2 | CF, cystic fibrosis | CSNK2, casein kinase 2 | CFTR, cystic fibrosis transmembrane conductance regulator | EGCG, epigallocatechin gallate | SQSTM1, sequestosome 1 | CXCL8, chemokine (C-X-C motif) ligand 8 | cystic fibrosis | cysteamine | sweat chloride | FEV, forced expiratory volume | RPD, rectal potential difference | CFTR | BECN1/Beclin 1, autophagy-related | Sweat chloride | Cysteamine | Cystic fibrosis | Epigallocatechin gallate | Autophagy | Life Sciences & Biomedicine | Science & Technology | Cell Biology | Tumor Necrosis Factor-alpha - metabolism | Sequestosome-1 Protein | Humans | Male | Chlorides - chemistry | Young Adult | Adult | Cystamine - pharmacology | Female | Cell Membrane - metabolism | Membrane Proteins - metabolism | Interleukin-8 - metabolism | Catechin - pharmacology | Child | Cysteamine - administration & dosage | Beclin-1 | Mice, Inbred CFTR | Administration, Oral | Cystic Fibrosis - metabolism | Mice, Transgenic | Apoptosis Regulatory Proteins - metabolism | Homozygote | Phenotype | Pilot Projects | Animals | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Catechin - analogs & derivatives | Mice | Mutation | Adaptor Proteins, Signal Transducing - metabolism | Index Medicus
Journal Article
American journal of physiology. Lung cellular and molecular physiology, ISSN 1522-1504, 11/2018, Volume 315, Issue 5, pp. L846 - L857
Ivacaftor is the first drug to target directly defects in the cystic fibrosis transmembrane conductance regulator (CFTR), which causes cystic fibrosis (CF). To... 
CFTR potentiation | Cystic fibrosis | F508del-CFTR | Ivacaftor (VX-770) | CFTR chloride ion channel | Respiratory System | Physiology | Life Sciences & Biomedicine | Science & Technology | Index Medicus
Journal Article
Journal of cystic fibrosis, ISSN 1569-1993, 01/2020, Volume 19, Issue 1, pp. 68 - 79
Ivacaftor is the first in a class of drugs, CFTR modulators, that target the underlying defect in cystic fibrosis (CF). This long-term observational safety... 
CF disease progression | Ivacaftor | CFTR modulator | CFTR | CF complications | Life Sciences & Biomedicine | Respiratory System | Science & Technology | Index Medicus
Journal Article
Journal Article