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European Journal of Medicinal Chemistry, ISSN 0223-5234, 10/2019, Volume 180, pp. 430 - 448
Deletion of phenylalanine at position 508 (F508del) in the CFTR protein, is the most common mutation causing cystic fibrosis (CF). F508del causes misfolding... 
Cystic fibrosis | CFTR correctors | F508del-CFTR | Cystic fibrosis transmembrane conductance regulator | CFTR | Index Medicus
Journal Article
P and T, ISSN 1052-1372, 2014, Volume 39, Issue 7, pp. 500 - 511
Defects in a single gene lead to the defective proteins that cause cystic fibrosis, making the disease an ideal candidate for mutation-targeted therapy.... 
CFTR potentiators | Ivacaftor | 4PBA | Cystic fibrosis transmembrane conductance regulator (CFTR) | VRT-532 | CFTR modulators | VX-661 | Cystic fibrosis | Ataluren | CFTR correctors | Lumacaftor | CFTR | cystic fibrosis transmembrane conductance regulator (CFTR) | cystic fibrosis | ataluren | lumacaftor | ivacaftor
Journal Article
American Journal of Respiratory and Critical Care Medicine, ISSN 1073-449X, 01/2018, Volume 197, Issue 2, pp. 214 - 224
Rationale: Tezacaftor (formerly VX-661) is an investigational small molecule that improves processing and trafficking of the cystic fibrosis transmembrane... 
Sweat chloride | Cystic fibrosis transmembrane conductance regulator corrector | Forced expiratory volume | CFTR modulator | EFFICACY | SAFETY | LUMACAFTOR-IVACAFTOR | IDENTIFICATION | INVESTIGATIONAL CFTR CORRECTOR | TRANSMEMBRANE CONDUCTANCE REGULATOR | G551D MUTATION | F508DEL-CFTR MUTATION | GENE | RESPIRATORY SYSTEM | cystic fibrosis transmembrane conductance regulator corrector | PHE508DEL CFTR | forced expiratory volume | sweat chloride | CRITICAL CARE MEDICINE | Prognosis | Humans | Male | Benzodioxoles - administration & dosage | Aminophenols - adverse effects | Indoles - administration & dosage | Dose-Response Relationship, Drug | Young Adult | Quinolones - adverse effects | Adult | Benzodioxoles - adverse effects | Female | Drug Therapy, Combination | Molecular Targeted Therapy - methods | Severity of Illness Index | Double-Blind Method | Drug Administration Schedule | Risk Assessment | Administration, Oral | Treatment Outcome | Maximum Tolerated Dose | Quinolones - administration & dosage | Indoles - adverse effects | Cystic Fibrosis - genetics | Adolescent | Cystic Fibrosis - diagnosis | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Respiratory Function Tests | Aminophenols - administration & dosage | Cystic Fibrosis - drug therapy | Proteins | Pediatrics | Chloride | Cysts | Cystic fibrosis | Mutation | Kinases | Patients | Drug dosages | Index Medicus | Abridged Index Medicus | Original
Journal Article
06/2009
The intrinsically disordered nonphosphorylated and phosphorylated R region of CFTR and its interactions with NBD1 and SLC26A3 STAS have been characterized at... 
disordered proteins | 0487 | CFTR
Dissertation
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, ISSN 1040-0605, 07/2019, Volume 317, Issue 1, pp. L71 - L86
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 11/2017, Volume 377, Issue 21, pp. 2013 - 2023
Patients with homozygous Phe508del cystic fibrosis were assigned to receive combination tezacaftor–ivacaftor or placebo for 24 weeks. The combination resulted... 
G551D MUTATION | MEDICINE, GENERAL & INTERNAL | IN-VITRO | LUMACAFTOR | EFFICACY | SAFETY | AGED 6-11 YEARS | F508DEL-CFTR | PHASE-3 | CFTR POTENTIATOR | COMBINATION | Proteins | Clinical trials | Cystic fibrosis | Mutation | Drug therapy | Patients | Drug dosages | Quality of life | Pharmaceuticals | Index Medicus | Abridged Index Medicus
Journal Article
Journal of Cystic Fibrosis, ISSN 1569-1993, 05/2017, Volume 16, Issue 3, pp. 371 - 379
Cavosonstat (N91115), an orally bioavailable inhibitor of S-nitrosoglutathione reductase, promotes cystic fibrosis transmembrane conductance regulator (CFTR)... 
GSNOR, F508del-CFTR | CFTR stabilizer | Cystic fibrosis | Modulator | Homozygous | DELTA-F508-CFTR | S-NITROSOGLUTATHIONE REDUCTASE | F508del-CFTR | IVACAFTOR | GSNOR | CORRECTORS | REGULATOR EXPRESSION | CFTR POTENTIATOR | MATURATION | LUMACAFTOR | EPITHELIAL-CELLS | RESPIRATORY SYSTEM | MUTATION | Safety and security measures | Index Medicus
Journal Article
03/2014
Cystic Fibrosis (CF) is a life-threatening autosomal recessive disease affecting 1:3600 children born in Canada. CF is caused by mutations in the Cystic... 
0379 | Cystic Fibrosis | 0719 | 0419 | phosphorylation | CFTR
Dissertation
01/2014
Objective: Cystic fibrosis (CF) is a common autosomal recessive genetic disease caused by a mutation in the CF transmembrane conductance regulatory (CFTR )... 
CFTR Gene Mutations | Cystic Fibrosis | Clinical Presentation
Web Resource
Current Drug Targets, ISSN 1389-4501, 09/2016, Volume 17, Issue 11, pp. 1275 - 1281
Journal Article
Journal Article
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, ISSN 1569-1993, 06/2019
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... 
Journal Article
JOURNAL OF CYSTIC FIBROSIS, ISSN 1569-1993, 03/2017, Volume 16, Issue 2, pp. 246 - 249
In an open-label, single-center phase 1 pharmacokinetic study in healthy subjects who received lumacaftor (LUM) in combination with ivacaftor (IVA), review of... 
IVACAFTOR | RESPIRATORY SYSTEM | PHE508DEL CFTR | MUTATION | CYSTIC-FIBROSIS | CFTR POTENTIATOR
Journal Article
1998, Methods in enzymology, ISBN 0121821935, Volume 292., xxxv, 853
Book
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, ISSN 0925-4439, 06/2019, Volume 1865, Issue 6, pp. 1323 - 1331
Background: We analyzed the CFTR response to VX-809/VX-770 drugs in conditionally reprogrammed cells (CRC) of human nasal epithelium (HNE) from F508de1/F508del... 
DIAGNOSIS | TEZACAFTOR-IVACAFTOR | BIOCHEMISTRY & MOLECULAR BIOLOGY | Cystic fibrosis | Ussing chamber | Modifier genes | CFTR POTENTIATOR | IDENTIFICATION | CRC-FINE | TRANSMEMBRANE CONDUCTANCE REGULATOR | LUMACAFTOR | BIOPHYSICS | CFTR modulator drugs | CLINICAL-TRIALS | IN-VIVO | LUNG-DISEASE | THERAPIES | SLC26A9 | CFTR
Journal Article
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