Effects of Lumacaftor–Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis
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Title
Effects of Lumacaftor–Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis
Authors
Keywords
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Journal
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 197, Issue 11, Pages 1433-1442
Publisher
American Thoracic Society
Online
2018-01-12
DOI
10.1164/rccm.201710-1983oc
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Note: Only part of the references are listed.- CORK Study in Cystic Fibrosis
- (2018) Nicola J. Ronan et al. CHEST
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- (2017) Meredith C. Fidler et al. Journal of Cystic Fibrosis
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- (2017) Steven M. Rowe et al. NEW ENGLAND JOURNAL OF MEDICINE
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- (2017) Felix Ratjen et al. Lancet Respiratory Medicine
- Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study
- (2017) Michael W Konstan et al. Lancet Respiratory Medicine
- Sources of Variation in Sweat Chloride Measurements in Cystic Fibrosis
- (2016) Joseph M. Collaco et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Cystic fibrosis
- (2016) J Stuart Elborn LANCET
- Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor
- (2015) Simon Y. Graeber et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR
- (2015) Claire E. Wainwright et al. NEW ENGLAND JOURNAL OF MEDICINE
- Cystic fibrosis
- (2015) Felix Ratjen et al. Nature Reviews Disease Primers
- Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis
- (2014) Steven M. Rowe et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- CFTR: cystic fibrosis and beyond
- (2014) M. A. Mall et al. EUROPEAN RESPIRATORY JOURNAL
- 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
- (2014) Michael P Boyle et al. Lancet Respiratory Medicine
- Sweat chloride as a biomarker of CFTR activity: Proof of concept and ivacaftor clinical trial data
- (2013) Frank J. Accurso et al. Journal of Cystic Fibrosis
- Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation
- (2013) Steven M. Rowe et al. PLoS One
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- (2013) John P. Clancy et al. PLoS One
- Multi-ethnic reference values for spirometry for the 3–95-yr age range: the global lung function 2012 equations
- (2012) Philip H. Quanjer et al. EUROPEAN RESPIRATORY JOURNAL
- A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation
- (2011) Bonnie W. Ramsey et al. NEW ENGLAND JOURNAL OF MEDICINE
- The K+ Channel Opener 1-EBIO Potentiates Residual Function of Mutant CFTR in Rectal Biopsies from Cystic Fibrosis Patients
- (2011) Eva K. Roth et al. PLoS One
- Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
- (2011) F. Van Goor et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation
- (2011) J P Clancy et al. THORAX
- Clinical Phenotype and Genotype of Children with Borderline Sweat Test and Abnormal Nasal Epithelial Chloride Transport
- (2010) Isabelle Sermet-Gaudelus et al. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
- Functional analysis of F508del CFTR in native human colon
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- Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation
- (2010) Frank J. Accurso et al. NEW ENGLAND JOURNAL OF MEDICINE
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