Development of elexacaftor - tezacaftor – ivacaftor: highly effective CFTR modulation for the majority of people with cystic fibrosis

Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators – an international survey

(2020) Edward F Nash et al.   Journal of Cystic Fibrosis

Testicular pain following initiation of elexacaftor/tezacaftor/ivacaftor in males with cystic fibrosis

(2020) Shannon M. Rotolo et al.   Journal of Cystic Fibrosis

Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations

(2020) Anne Munck et al.   Journal of Cystic Fibrosis

Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis

(2020) Julie Safirstein et al.   Journal of Cystic Fibrosis

Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis

(2020) Emily DiMango et al.   Journal of Cystic Fibrosis

Azithromycin and tezacaftor/ivacaftor is associated with first-degree heart block in an adult with cystic fibrosis

(2020) Yang Song et al.   Journal of Cystic Fibrosis

Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for ≥24 Weeks in People With CF and ≥1 F508del Allele: Interim Results of an Open-Label Phase Three Clinical Trial

(2020) Matthias Griese et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis

(2019) Xingshen Sun et al.   Science Translational Medicine

ERS/TSANZ Task Force Statement on the Management of Reproduction and Pregnancy in Women with Airways Diseases

(2019) Peter G. Middleton et al.   EUROPEAN RESPIRATORY JOURNAL

Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial

(2019) Harry G M Heijerman et al.   LANCET

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

(2019) Peter G. Middleton et al.   NEW ENGLAND JOURNAL OF MEDICINE

VX-445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

(2018) Dominic Keating et al.   NEW ENGLAND JOURNAL OF MEDICINE

Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

(2017) Steven M. Rowe et al.   NEW ENGLAND JOURNAL OF MEDICINE

Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

(2017) Jennifer L. Taylor-Cousar et al.   NEW ENGLAND JOURNAL OF MEDICINE

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

(2016) Gudio Veit et al.   MOLECULAR BIOLOGY OF THE CELL

Classification of CFTR mutation classes

(2016) Fernando Augusto Lima Marson et al.   Lancet Respiratory Medicine

Progress in therapies for cystic fibrosis

(2016) Kris De Boeck et al.   Lancet Respiratory Medicine

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Ivacaftor in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation

(2012) Patrick A. Flume et al.   CHEST

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent Manner

(2012) Paul D. W. Eckford et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

Determination of the Minimal Clinically Important Difference Scores for the Cystic Fibrosis Questionnaire-Revised Respiratory Symptom Scale in Two Populations of Patients With Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection

(2009) Alexandra L. Quittner et al.   CHEST

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA