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Journal of Cystic Fibrosis, ISSN 1569-1993, 09/2018, Volume 17, Issue 5, pp. 595 - 606
Current modulator therapies for some cystic fibrosis-causing CFTR mutants, including N1303K, have limited efficacy. We provide evidence here to support... 
High-throughput screen | Cystic fibrosis | N1303K | CFTR | Potentiator | CORRECTORS | ORGANOIDS | TRANSMEMBRANE CONDUCTANCE REGULATOR | IN-VITRO | RESCUE | RESPIRATORY SYSTEM | SYNERGY | VX-809 | CYSTIC-FIBROSIS | MUTATIONS | G551D | Care and treatment | Flavonoids | Flavones | Bioflavonoids | high-throughput screen | potentiator
Journal Article
Journal of General Physiology, ISSN 0022-1295, 12/2017, Volume 149, Issue 12, pp. 1105 - 1118
Cystic fibrosis (CF) is a channelopathy caused by loss-of-function mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which... 
CHLORIDE-CHANNEL | ATP HYDROLYSIS | IN-VITRO | DOMAIN | PHYSIOLOGY | SIDE-CHAIN | ION-CHANNEL | CYSTIC-FIBROSIS MUTATION | CL-CHANNEL | CONDUCTANCE-REGULATOR CFTR | BINDING | Cystic fibrosis | Ion channels | Research | Adenosine triphosphate | 514
Journal Article
Current Opinion in Pharmacology, ISSN 1471-4892, 06/2017, Volume 34, pp. 98 - 104
One major breakthrough in cystic fibrosis research in the past decade is the development of drugs that target the root cause of the disease — dysfunctional... 
GATING DEFECTS | TRANSMEMBRANE-CONDUCTANCE-REGULATOR | IN-VITRO | NUCLEOTIDE-BINDING DOMAINS | TYROSINE KINASE | PHE508DEL CFTR | ATP BINDING | PHARMACOLOGY & PHARMACY | CL-CHANNEL | CYSTIC-FIBROSIS | CHLORIDE CHANNEL | Medical colleges | Cystic fibrosis | Analysis | Resveratrol
Journal Article
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, ISSN 1040-0605, 07/2019, Volume 317, Issue 1, pp. L71 - L86
Journal Article
Journal Article
American journal of respiratory and critical care medicine, ISSN 1073-449X, 2014, Volume 190, Issue 2, pp. 175 - 184
Rationale: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator recently approved for patients with CF age 6 and older with... 
Cystic fibrosis | Pseudomonas aeruginosa | Ivacaftor | CFTR modulator | LUNG-FUNCTION | ivacaftor | MUCIN SECRETION | ADRENERGIC SWEAT SECRETION | MUCOCILIARY CLEARANCE | INTESTINAL PH | CHILDREN | RESPIRATORY SYSTEM | cystic fibrosis | PSEUDOMONAS-AERUGINOSA | INFLAMMATION | DISEASE | pH | QUESTIONNAIRE | CRITICAL CARE MEDICINE | Sputum - microbiology | Lung - microbiology | Pseudomonas aeruginosa - isolation & purification | Follow-Up Studies | Forced Expiratory Volume - drug effects | Sweat - drug effects | Humans | Microbiota - drug effects | Respiratory System Agents - therapeutic use | Hospitalization - statistics & numerical data | Male | Aminophenols - therapeutic use | Quinolones - pharmacology | Aminophenols - pharmacology | Mucociliary Clearance - drug effects | Pseudomonas Infections - prevention & control | Young Adult | Hydrogen-Ion Concentration - drug effects | Quinolones - therapeutic use | Adult | Female | Lung - metabolism | Child | Biomarkers - metabolism | Respiratory System Agents - pharmacology | Cystic Fibrosis - metabolism | Cystic Fibrosis - microbiology | Pseudomonas Infections - complications | Pseudomonas Infections - diagnosis | Treatment Outcome | Lung - physiopathology | Genetic Markers | Sputum - metabolism | Cystic Fibrosis - genetics | Intestine, Small - drug effects | Lung - drug effects | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Intestine, Small - metabolism | Mutation | Cystic Fibrosis - drug therapy | Sweat - metabolism | Original
Journal Article
Autophagy, ISSN 1554-8627, 11/2012, Volume 8, Issue 11, pp. 1657 - 1672
Channel activators (potentiators) of cystic fibrosis (CF) transmembrane conductance regulator (CFTR), can be used for the treatment of the small subset of CF... 
cystamine | CFTR potentiators | autophagy | cystic fibrosis | therapy | Binding | Proteins | Landes | Calcium | Bioscience | Biology | Cell | Cycle | Cancer | Organogenesis | Therapy | Cystic fibrosis | Autophagy | Cystamine | GAMMA DOWN-REGULATION | OXIDATIVE STRESS | F508DEL-CFTR | PLASMA-MEMBRANE | INTESTINAL EPITHELIAL-CELLS | CELL BIOLOGY | PROTEIN-QUALITY CONTROL | MUTATION | TISSUE TRANSGLUTAMINASE | LUNG INFLAMMATION | CFTR | Inflammation - pathology | Epithelial Cells - metabolism | Sequestosome-1 Protein | Epithelial Cells - drug effects | Humans | Cystic Fibrosis - pathology | Male | Nasal Mucosa - pathology | Molecular Targeted Therapy | Autophagy - drug effects | Salicylates - pharmacology | Cystamine - therapeutic use | Organometallic Compounds - therapeutic use | Cystamine - pharmacology | Female | Cell Membrane - metabolism | Membrane Proteins - metabolism | Child | Cell Membrane - drug effects | Nasal Mucosa - drug effects | Beclin-1 | Epithelium - drug effects | Epithelium - pathology | Lung - pathology | Nasal Polyps - pathology | Epithelium - metabolism | Heat-Shock Proteins - metabolism | Epithelial Cells - pathology | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Antioxidants - pharmacology | Salicylates - therapeutic use | Apoptosis Regulatory Proteins - metabolism | Animals | Lung - drug effects | Adolescent | Genistein - pharmacology | Lipopolysaccharides - pharmacology | Mice | Adaptor Proteins, Signal Transducing - metabolism | Cystic Fibrosis - drug therapy | Organometallic Compounds - pharmacology | Translational Research Paper
Journal Article