chlorine and vx-770

chlorine has been researched along with vx-770 in 52 studies

Research

Studies (52)

Timeframe	Studies, this research(%)	All Research%
pre-1990	0 (0.00)	18.7374
1990's	0 (0.00)	18.2507
2000's	1 (1.92)	29.6817
2010's	33 (63.46)	24.3611
2020's	18 (34.62)	2.80

Authors

Authors	Studies
Arumugam, V; Ashlock, M; Burton, B; Cao, D; Decker, C; Frizzell, RA; Grootenhuis, PD; Hadida, S; Hazlewood, A; Joubran, J; McCartney, J; Negulescu, P; Neuberger, T; Olson, ER; Singh, A; Turnbull, A; Van Goor, F; Wine, JJ; Yang, J; Young, C; Zhou, J	1
Accurso, FJ; Aitken, ML; Ashlock, MA; Boyle, MP; Campbell, PW; Clancy, JP; Donaldson, SH; Dong, Q; Dunitz, JM; Durie, PR; Freedman, SD; Hornick, DB; Konstan, MW; Mayer-Hamblett, N; Moss, RB; Olson, ER; Ordoñez, CL; Pilewski, JM; Ramsey, BW; Rose, LM; Rowe, SM; Rubenstein, RC; Sagel, SD; Stone, AJ; Uluer, AZ; Zha, J	1
Borowitz, DS; Flume, PA; Geller, DE; Li, H; Liou, TG; Ordoñez, CL; Yen, K	1
Chowdhury, BA; Durmowicz, AG; Rosebraugh, CJ; Witzmann, KA	1
Sermet-Gaudelus, I	1
Elborn, JS	1
Mueller, P; Rodman, D; Schmelz, A; Seliger, VI; Van Goor, F	1
Burton, B; Hoffman, BJ; Van Goor, F; Yu, H	1
Accurso, FJ; Beamer, JR; Clancy, JP; Cohen, M; Dong, Q; Hathorne, H; Hill, A; Liu, B; Olson, ER; Ordoñez, CL; Rowe, SM; Stone, AJ	1
Harrison, MJ; Murphy, DM; Plant, BJ	1
McGarry, ME; Nielson, DW	1
Baasov, T; Bedwell, DM; Belakhov, V; Biswas, S; Chen, F; Dai, Y; Du, M; Hong, J; J Bridges, R; Jackson, LA; Mutyam, V; Rowe, SM; Schacht, J; Shalev, M; Tang, L; Xue, X	1
Barry, PJ; Horsley, AR; Jones, AM; Webb, AK	1
Grasemann, H; Ratjen, F; Solomon, M	1
Accurso, FJ; Ashlock, MA; Campbell, PW; Clancy, JP; Dong, Q; Heltshe, SL; Hoch, HE; Konstan, MW; Ordonez, CL; Ramsey, BW; Rowe, SM; Stone, AJ; Van Goor, F; Zha, J	1
Bergin, DA; Clynes, M; Hayes, E; Henry, M; Jundi, B; Keenan, J; McCarthy, C; McElvaney, NG; McElvaney, OJ; Meleady, P; Molloy, K; Pohl, K; Reeves, EP; White, MM	1
Bell, SC; Boyle, MP; Huang, X; Konstan, MW; McColley, SA; Patel, NR; Rietschel, E; Rodman, D; Rowe, SM; Waltz, D	1
Apaja, PM; Avramescu, RG; Bagdany, M; Borot, F; Finkbeiner, WE; Hegedus, T; Lukacs, GL; Perdomo, D; Phuan, PW; Szollosi, D; Veit, G; Verkman, AS; Wu, YS	1
Ahrens, R; Elborn, JS; Johnson, C; Konstan, MW; Munck, A; Plant, BJ; Rodriguez, S	1
Cooke, J; Cunningham, S; Davies, JC; Green, Y; Harris, WT; Lapey, A; Regelmann, WE; Robertson, S; Rosenfeld, M; Sawicki, GS; Southern, KW	1
Bilton, D; Davies, JC; De Boeck, K; Le Camus, C; Milenković, D; Vermeulen, F	1
Beekman, JM; Berkers, G; Bronsveld, I; Clevers, H; de Jonge, HR; de Rijke, YB; de Winter-de Groot, KM; Dekkers, JF; Escher, JC; Heijerman, HG; Houwen, RH; Janssens, HM; Kruisselbrink, E; Majoor, CJ; Nieuwenhuis, EE; van de Graaf, EA; van der Ent, CK; Vleggaar, FP; Vonk, A	1
Bassinet, L; Cohen, M; Escabasse, V; Friedman, H; Hatton, A; Le Bourgeois, M; Livnat, G; Lucidi, V; Mesbahi, M; Nguyen-Khoa, T; Sermet-Gaudelus, I; Shteinberg, M; Wilschanski, M	1
Beusmans, J; Fidler, MC; Panorchan, P; Van Goor, F	1
Finkbeiner, WE; Illek, B; Ly, NP; McGarry, ME; Moreno, C; Nielson, DW; Olshansky, S; Zlock, L	1
Casavola, V; Castellani, S; Conese, M; D'Oria, S; Favia, M; Gallo, C; Guerra, L; Leonetti, G; Manca, A; Mariggiò, MA; Montemurro, P; Polizzi, AM; Santostasi, T	1
Arnold, C; Hentschel, J; Mainz, JG; Tabori, H	1
Abou Alaiwa, MH; Carter, S; Grogan, B; Launspach, JL; McKone, EF; Singh, PK; Stoltz, DA; Welsh, MJ; Zabner, J	1
Burr, L; Daines, C; Keating, D; Mall, MA; Marigowda, G; McKee, CM; McKone, EF; Moskowitz, SM; Ramsey, BW; Robertson, S; Rowe, SM; Sass, LA; Savage, J; Simard, C; Taylor-Cousar, JL; Tullis, E; Van Goor, F; Waltz, D; Xuan, F; Young, T	1
Brown, C; Davies, JC; Horsley, A; Mall, MA; McKee, CM; McKone, EF; Moskowitz, SM; Plant, BJ; Prais, D; Ramsey, BW; Robertson, S; Rowe, SM; Shilling, RA; Simard, C; Taylor-Cousar, JL; Tullis, E; Uluer, A; Van Goor, F; Waltz, D; Xuan, F; Young, T	1
Dunn, C; Farahmand, M; Horii, RI; Kim, J; Milla, CE; Moss, RB; Thomas, EAC; Wine, JJ	1
Course, CW; Hanks, R	1
Braggion, C; Carnovale, V; Castellani, C; Cimino, G; Colangelo, C; Ferrara, N; Francalanci, M; Iacotucci, P; Leonetti, G; Lucidi, V; Manca, A; Salvatore, D; Vitullo, P	1
Dřevínek, P; Jain, R; Lands, LC; Mall, MA; Marigowda, G; McKee, CM; McKone, EF; Middleton, PG; Moskowitz, SM; Nair, N; Polineni, D; Ramsey, BW; Rowe, SM; Savage, J; Simard, C; Taylor-Cousar, JL; Tian, S; Tullis, E; Vermeulen, F; Waltz, D; Xuan, F	1
Guimbellot, JS; Heltshe, SL; Jain, M; Jovanovic, B; Rowe, SM; Sagel, SD; Secunda, KE	1
Bridges, RJ; Hastings, ML; Michaels, WE	1
Chen, J; Conrath, K; Falk Libby, E; Li, Y; Mutyam, V; Peng, N; Rowe, SM; Sharma, J; Singh, AK; Tang, LP	1
Amato, F; Castaldo, G; Castellani, C; Ferrari, B; Galietta, LJV; Taccetti, G; Terlizzi, V	1
Dittrich, AM; Hansen, G; Junge, S; Nietert, M; Pallenberg, ST; Ringshausen, FC; Sauer-Heilborn, A; Tümmler, B	1
Bijvelds, MJC; de Jonge, HR; Janssens, HM; Meijsen, KF; Roest, HP; Roos, FJM; van der Laan, LJW; Verstegen, MMA	1
Branstetter, BF; Goralski, JL; Kimple, AJ; Lee, SE; Nouraie, SM; Pilewski, JM; Senior, BA; Shaffer, AD; Stapleton, AL; Zemke, AC	1
Aksit, MA; Bridges, RJ; Cutting, GR; Faino, AV; Gibson, RL; Goralski, JL; Ling, H; Merlo, CA; Montemayor, K; Onchiri, FM; Osorio, DL; Patel, SU; Paul, KC; Raraigh, KS; Sciortino, S; Sharma, N; Sheppard, DN; Thakerar, A; Wang, Y; West, NE; Worthington, EN	1
Clifton, IJ; Etherington, C; Gillgrass, L; Peckham, DG; Pollard, K; Shaw, N; Spoletini, G; Williams, E	1
Konstan, MW; Mayer-Hamblett, N; Odem-Davis, K; Rowe, SM; VanDevanter, D; Warden, M; Young, J; Zemanick, ET	1
Baroni, D; Ludovico, A; Moran, O	1
Amer, AO; Bai, S; Brown, N; Carey, K; Hall-Stoodley, L; Jaganathan, D; Kopp, BT; McCoy, KS; Partida-Sanchez, S; Pham, H; Robledo-Avila, F; Shrestha, CL; Wisniewski, BL; Zhang, S	1
Ilivitzki, A; Khatib, M; Koren, I; Livnat, G; Schnapp, Z; Shorbaji, N; Shteinberg, M; Yaacoby-Bianu, K	1
Cohen-Cymberknoh, M; Dagan, A; Gur, M; Heching, M; Livnat, G; Mei-Zahav, M; Prais, D; Shmueli, E; Shteinberg, M; Stein, N; Yaacoby-Bianu, K	1
Foucaud, P; Mercier, JC	1
Durieu, I; Durupt, S; Reynaud, Q	1
Azevedo, P; Bolas, R; Custódio, C; Lopes, C; Lopes, K	1
Blanchard, SC; Chen, J; Fitzgerald, G; Kilic, Z; Levring, J; Terry, DS	1

Reviews

3 review(s) available for chlorine and vx-770

Article	Year
Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation. European respiratory review : an official journal of the European Respiratory Society, 2013, Mar-01, Volume: 22, Issue:127 Topics: Adolescent; Adult; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Humans; Lung; Molecular Targeted Therapy; Mutation; Patient Selection; Phenotype; Precision Medicine; Quinolones; Respiratory System Agents; Sweat; Time Factors; Treatment Outcome; Young Adult	2013
Newborn screening for cystic fibrosis: Is there benefit for everyone? Paediatric respiratory reviews, 2019, Volume: 31 Topics: Aminophenols; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; False Positive Reactions; Health Policy; Humans; Infant, Newborn; Mutation; Neonatal Screening; Penetrance; Phenotype; Quinolones; Risk Assessment; Sweat	2019
[Therapeutic advances in cystic fibrosis: from genetics to treatment personalized]. La Revue du praticien, 2022, Volume: 72, Issue:8 Topics: Aminophenols; Anti-Bacterial Agents; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Quality of Life; Sweat	2022

Trials

14 trial(s) available for chlorine and vx-770

Article	Year
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. The New England journal of medicine, 2010, Nov-18, Volume: 363, Issue:21 Topics: Adult; Aminophenols; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Forced Expiratory Volume; Humans; Ion Channels; Male; Membrane Potentials; Middle Aged; Mutation; Nasal Mucosa; Quinolones; Sweat; Young Adult	2010
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. Chest, 2012, Volume: 142, Issue:3 Topics: Adolescent; Adult; Alleles; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Female; Homozygote; Humans; Male; Middle Aged; Mutation; Pharmacogenetics; Quinolones; Sweat; Treatment Outcome; Young Adult	2012
Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation. PloS one, 2013, Volume: 8, Issue:7 Topics: Adult; Amino Acid Substitution; Aminophenols; Biological Transport; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Demography; Epithelial Sodium Channels; Female; Humans; Male; Membrane Potentials; Middle Aged; Mutation; Nose; Placebos; Quinolones; Sample Size; Sodium; Solutions; Young Adult	2013
Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:2 Topics: Adult; Aminophenols; Biomarkers; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Double-Blind Method; Drug Monitoring; Female; Humans; Male; Mutation; Nasal Mucosa; Quinolones; Reproducibility of Results; Respiratory System Agents; Specimen Handling; Sweat; Treatment Outcome	2014
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial. The Lancet. Respiratory medicine, 2014, Volume: 2, Issue:7 Topics: Adolescent; Adult; Aminophenols; Aminopyridines; Base Sequence; Benzodioxoles; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Therapy, Combination; Female; Forced Expiratory Volume; Heterozygote; Homozygote; Humans; Male; Quinolones; Sequence Deletion; Sweat; Young Adult	2014
Efficacy response in CF patients treated with ivacaftor: post-hoc analysis. Pediatric pulmonology, 2015, Volume: 50, Issue:5 Topics: Adolescent; Adult; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Female; Forced Expiratory Volume; Humans; Male; Mutation; Numbers Needed To Treat; Quinolones; Respiratory Function Tests; Surveys and Questionnaires; Sweat; Weight Gain; Young Adult	2015
Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. The Lancet. Respiratory medicine, 2016, Volume: 4, Issue:2 Topics: Aminophenols; Child, Preschool; Chloride Channel Agonists; Chlorides; Cough; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Genotype; Humans; Male; Mutation; Quinolones; Sweat	2016
Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1 Topics: Adolescent; Adult; Aminophenols; Biological Variation, Population; Biomarkers; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Mutation; Quinolones; Retrospective Studies; Sweat	2017
In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies. Pediatric pulmonology, 2017, Volume: 52, Issue:4 Topics: Administration, Oral; Adolescent; Adult; Aminophenols; Chloride Channel Agonists; Chlorides; Cross-Over Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Middle Aged; Quinolones; Sweat; Treatment Outcome; Young Adult	2017
CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy. Pediatric pulmonology, 2017, Volume: 52, Issue:7 Topics: Adolescent; Adult; Aminophenols; C-Reactive Protein; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Leukocytes, Mononuclear; Male; Mutation; Neutrophils; Quinolones; Respiratory Function Tests; Sweat; Young Adult	2017
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. The New England journal of medicine, 2018, 10-25, Volume: 379, Issue:17 Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Genotype; Humans; Indoles; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat; Young Adult	2018
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. The New England journal of medicine, 2018, 10-25, Volume: 379, Issue:17 Topics: Adolescent; Adult; Alleles; Aminophenols; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Genotype; Humans; Indoles; Male; Mutation; Pyrazoles; Pyrrolidines; Quinolones; Sweat; Young Adult	2018
Sweat rate analysis of ivacaftor potentiation of CFTR in non-CF adults. Scientific reports, 2018, 11-02, Volume: 8, Issue:1 Topics: Adult; Aminophenols; Chloride Channel Agonists; Chlorides; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Female; Humans; Male; Quinolones; Sweat; Sweat Glands; Sweating	2018
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. The New England journal of medicine, 2019, 11-07, Volume: 381, Issue:19 Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Double-Blind Method; Drug Combinations; Female; Forced Expiratory Volume; Genotype; Humans; Indoles; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat; Young Adult	2019

Other Studies

35 other study(ies) available for chlorine and vx-770

Article	Year
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proceedings of the National Academy of Sciences of the United States of America, 2009, Nov-03, Volume: 106, Issue:44 Topics: Absorption; Amino Acid Substitution; Aminophenols; Animals; Bronchi; Cells, Cultured; Chlorides; Cilia; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Synergism; Epithelial Cells; Epithelial Sodium Channels; Humans; Ion Channel Gating; Mice; Mutation; NIH 3T3 Cells; Quinolines; Quinolones; Sodium	2009
Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience. Chest, 2013, Volume: 143, Issue:1 Topics: Aminophenols; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Forced Expiratory Volume; Humans; Outcome Assessment, Health Care; Quinolones; Sweat; Treatment Outcome	2013
The impact of personalised therapies on respiratory medicine. European respiratory review : an official journal of the European Respiratory Society, 2013, Mar-01, Volume: 22, Issue:127 Topics: Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Humans; Lung; Molecular Targeted Therapy; Mutation; Patient Selection; Phenotype; Precision Medicine; Pulmonary Medicine; Quinolones; Respiratory System Agents; Sweat; Treatment Outcome	2013
The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2013, Volume: 12, Issue:6 Topics: Administration, Oral; Adolescent; Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Male; Mutation; Quinolones; Sensitivity and Specificity; Sweat; Weight Gain	2013
Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:1 Topics: Aminophenols; Animals; Cell Line; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; In Vitro Techniques; Ion Channel Gating; Mutation, Missense; Quinolones; Rats; Rats, Inbred F344; Thyroid Gland	2014
Ivacaftor in a G551D homozygote with cystic fibrosis. The New England journal of medicine, 2013, Sep-26, Volume: 369, Issue:13 Topics: Adolescent; Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Homozygote; Humans; Mutation; Quinolones; Sweat	2013
Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N. Chest, 2013, Volume: 144, Issue:4 Topics: Aminophenols; Child; Chlorides; Cystic Fibrosis; Female; Humans; Mutation; Quinolones; Sweat	2013
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor. American journal of respiratory cell and molecular biology, 2014, Volume: 50, Issue:4 Topics: Aminoglycosides; Aminophenols; Animals; Biological Transport; Cell Line; Chlorides; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Drug Synergism; Genes, Reporter; Humans; Luciferases; Mice; Mice, Inbred CFTR; Mice, Transgenic; Organ of Corti; Quinolones; Rats; Rats, Inbred F344; Time Factors; Transfection	2014
Sweat chloride is not a useful marker of clinical response to Ivacaftor. Thorax, 2014, Volume: 69, Issue:6 Topics: Aminophenols; Biomarkers; Body Height; Body Weight; Chlorides; Cohort Studies; Cystic Fibrosis; Forced Expiratory Flow Rates; Humans; Lung; Prospective Studies; Quinolones; Spirometry; Sweat	2014
Aquagenic wrinkling of the palms in a patient with cystic fibrosis. The New England journal of medicine, 2013, Dec-12, Volume: 369, Issue:24 Topics: Aminophenols; Child; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Hand; Humans; Male; Quinolones; Skin Aging; Sweat; Water	2013
A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy. Blood, 2014, Aug-14, Volume: 124, Issue:7 Topics: Adult; Aminophenols; Cell Degranulation; Cells, Cultured; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electrophoresis, Gel, Two-Dimensional; Female; Homeostasis; Humans; Immunoblotting; Magnesium; Male; Mutation; Neutrophils; Protein Transport; Proteome; Proteomics; Quinolones; rab GTP-Binding Proteins; rab27 GTP-Binding Proteins; Sodium; Tumor Necrosis Factor-alpha; Young Adult	2014
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression. Science translational medicine, 2014, Jul-23, Volume: 6, Issue:246 Topics: Aminophenols; Bronchi; Cell Membrane; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Down-Regulation; Endocytosis; Epithelial Cells; Humans; Ion Channel Gating; Mutation; Quinolones; Suppression, Genetic; Time Factors	2014
Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis. Science translational medicine, 2016, 06-22, Volume: 8, Issue:344 Topics: Aminophenols; Aminopyridines; Benzodioxoles; Biological Assay; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Humans; In Vitro Techniques; Mutation; Organoids; Quinolones	2016
Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1 Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Male; Membrane Potentials; Middle Aged; Mutation; Nasal Mucosa; Outcome Assessment, Health Care; Quinolones; Sweat; Treatment Outcome	2017
Correlation of sweat chloride and percent predicted FEV Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017, Volume: 16, Issue:1 Topics: Aminophenols; Biomarkers; Chloride Channel Agonists; Chlorides; Clinical Trials as Topic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Forced Expiratory Volume; Humans; Mutation; Predictive Value of Tests; Quinolones; Sweat; Treatment Outcome	2017
Effects of Ivacaftor in Three Pediatric Siblings With Cystic Fibrosis Carrying the Mutations G551D And F508del. Archivos de bronconeumologia, 2018, Volume: 54, Issue:4 Topics: Adolescent; Aminophenols; Child, Preschool; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Ion Transport; Male; Mutation, Missense; Pancreas; Quinolones; Sequence Deletion; Treatment Outcome	2018
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis. JCI insight, 2018, 08-09, Volume: 3, Issue:15 Topics: Adult; Aminophenols; Animals; Bicarbonates; Biological Transport, Active; Bronchoalveolar Lavage Fluid; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Female; Humans; Hydrogen-Ion Concentration; Infant; Infant, Newborn; Ion Transport; Longitudinal Studies; Lung; Male; Middle Aged; Mutation; Quinolones; Respiratory Mucosa; Sweat; Young Adult	2018
Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations. Pediatric pulmonology, 2019, Volume: 54, Issue:9 Topics: Adolescent; Adult; Aminophenols; Child; Chloride Channel Agonists; Chlorides; Compassionate Use Trials; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Humans; Ion Channel Gating; Lung; Male; Middle Aged; Mutation; Quinolones; Respiratory Function Tests; Retrospective Studies; Sweat	2019
Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males. American journal of respiratory and critical care medicine, 2020, 04-15, Volume: 201, Issue:8 Topics: Adolescent; Adult; Age Factors; Aminophenols; Body Mass Index; Body Weight; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Disease Progression; Female; Forced Expiratory Volume; Humans; Male; Quinolones; Sex Factors; Sweat; Young Adult	2020
Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells. Nucleic acids research, 2020, 07-27, Volume: 48, Issue:13 Topics: Aminophenols; Bronchi; Cell Line, Tumor; Cells, Cultured; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Ion Transport; Mutation; Oligodeoxyribonucleotides, Antisense; Quinolones; RNA Splicing	2020
Novel Correctors and Potentiators Enhance Translational Readthrough in CFTR Nonsense Mutations. American journal of respiratory cell and molecular biology, 2021, Volume: 64, Issue:5 Topics: Aminophenols; Aminopyridines; Animals; Benzoates; Benzodioxoles; Benzopyrans; Cell Line; Chlorides; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Humans; Ion Transport; Protein Biosynthesis; Pyrans; Pyrazoles; Quinolones; Rats; Recovery of Function; Thyroid Epithelial Cells	2021
Ex vivo model predicted in vivo efficacy of CFTR modulator therapy in a child with rare genotype. Molecular genetics & genomic medicine, 2021, Volume: 9, Issue:4 Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Child, Preschool; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; Female; Genotype; Humans; Mutation, Missense; Nasal Mucosa; Pancreatic Elastase; Quinolones	2021
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3 Topics: Adrenergic Agents; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Indoles; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sweat	2022
Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:3 Topics: Aminophenols; Benzodioxoles; Bicarbonates; Chloride Channel Agonists; Chloride-Bicarbonate Antiporters; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Epithelial Cells; Humans; Indoles; Organoids; Pyrazoles; Pyridines; Pyrrolidines; Quinolones	2022
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:5 Topics: Adolescent; Adult; Aminophenols; Benzodioxoles; Child; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Indoles; Middle Aged; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Sinusitis; Young Adult	2022
CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor. JCI insight, 2022, 03-22, Volume: 7, Issue:6 Topics: Aminophenols; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Phenotype; Quinolones	2022
Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022, Volume: 21, Issue:6 Topics: Adult; Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mental Health; Mutation	2022
Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2023, Volume: 22, Issue:1 Topics: Aminophenols; Benzodioxoles; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Mutation; Sweat	2023
Modulator Combination Improves In Vitro the Microrheological Properties of the Airway Surface Liquid of Cystic Fibrosis Airway Epithelia. International journal of molecular sciences, 2022, Sep-27, Volume: 23, Issue:19 Topics: Aminophenols; Benzodioxoles; Bicarbonates; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Phenylalanine; Quinolones	2022
Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor. The European respiratory journal, 2023, Volume: 61, Issue:4 Topics: Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Macrophages; Mutation	2023
Real life evaluation of the multi-organ effects of Lumacaftor/Ivacaftor on F508del homozygous cystic fibrosis patients. BMC pharmacology & toxicology, 2022, 10-20, Volume: 23, Issue:1 Topics: Adolescent; Adult; Anti-Bacterial Agents; C-Peptide; Calcium; Chloride Channel Agonists; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Glucose; Humans; Mutation; Prospective Studies; Young Adult	2022
Treatment effects of Elexacaftor/Tezacaftor/Ivacaftor in people with CF carrying non-F508del mutations. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2023, Volume: 22, Issue:3 Topics: Aminophenols; Anti-Bacterial Agents; Benzodioxoles; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Retrospective Studies	2023
CFTR pharmacological modulators: A great advance in cystic fibrosis management. Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 2023, Volume: 30, Issue:1 Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Combinations; Humans; Infant, Newborn; Mutation; Quality of Life	2023
Elexacaftor/tezacaftor/ivacaftor-real-world clinical effectiveness and safety. A single-center Portuguese study. Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia, 2023, Volume: 49, Issue:2 Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Mutation; Portugal; Treatment Outcome	2023
CFTR function, pathology and pharmacology at single-molecule resolution. Nature, 2023, Volume: 616, Issue:7957 Topics: Adenosine Triphosphatases; Adenosine Triphosphate; Allosteric Regulation; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Electric Conductivity; Electrophysiology; Fluorescence Resonance Energy Transfer; Humans; Ion Channel Gating; Protein Multimerization	2023

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