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Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation

Journal of cystic fibrosis, 2018-03, Vol.17 (2), p.e13-e16

2018

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A small molecule CFTR potentiator restores ATP‐dependent channel gating to the cystic fibrosis mutant G551D‐CFTR

British journal of pharmacology, 2022-04, Vol.179 (7), p.1319-1337

2022

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Modulation, microbiota and inflammation in the adult CF gut: A prospective study

Journal of cystic fibrosis, 2022-09, Vol.21 (5), p.837-843

2022

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Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non- G551D gating mutation

Journal of cystic fibrosis, 2014-12, Vol.13 (6), p.674-680

2014

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CORK Study in Cystic Fibrosis

Chest, 2018-02, Vol.153 (2), p.395-403

2018

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Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation

American journal of respiratory and critical care medicine, 2013-06, Vol.187 (11), p.1219-1225

2013

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CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor

Chest, 2018-02, Vol.153 (2), p.395-403

2018

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Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR

American journal of respiratory and critical care medicine, 2020-11, Vol.202 (9), p.1271-1282

2020

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GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1)

Journal of cystic fibrosis, 2019-09, Vol.18 (5), p.693-699

2019

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IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation

Journal of cystic fibrosis, 2019-03, Vol.18 (2), p.286-293

2019

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Ivacaftor partially corrects airway inflammation in a humanized G551D rat

American journal of physiology. Lung cellular and molecular physiology, 2021-06, Vol.320 (6), p.L1093-L1100

2021

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Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis

Health technology assessment (Winchester, England), 2014-03, Vol.18 (18), p.1-106

2014

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On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: A molecular dynamic study

Biochimie, 2015-04, Vol.111, p.19-29

2015

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SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR

Journal of cystic fibrosis, 2021-09, Vol.20 (5), p.851-856

2021

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Pregnancy among cystic fibrosis women in the era of CFTR modulators

Journal of cystic fibrosis, 2017-11, Vol.16 (6), p.687-694

2017

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Improvement of Hepatic Steatosis in Cystic Fibrosis With Ivacaftor Therapy

Journal of pediatric gastroenterology and nutrition, 2015-05, Vol.60 (5), p.578-579

2015

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G551D mutation impairs PKA-dependent activation of CFTR channel that can be restored by novel GOF mutations

American journal of physiology. Lung cellular and molecular physiology, 2020-11, Vol.319 (5), p.L770-L785

2020

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Ivacaftor: Five‐year outcomes in the West of Scotland cystic fibrosis population

The clinical respiratory journal, 2023-05, Vol.17 (5), p.473-477

2023

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G551D-CFTR needs more bound actin than wild-type CFTR to maintain its presence in plasma membranes

Cell biology international, 2015-08, Vol.39 (8), p.978-985

2015

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Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations

Journal of cystic fibrosis, 2017-01, Vol.16 (1), p.36-40

2017

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Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor

Journal of cystic fibrosis, 2016-01, Vol.15 (1), p.67-73

2016

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Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations

eLife, 2019-06, Vol.8

2019

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Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies: Evaluation of: Accurso FJ, Rowe SM, Clancy JP, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010;363(21):1991-2003

Expert opinion on investigational drugs, 2011-03, Vol.20 (3), p.423-425

Antoniu, Sabina Antonela

2011

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Patient-reported outcomes in patients with cystic fibrosis with a G551D mutation on ivacaftor treatment: results from a cross-sectional study

BMC pulmonary medicine, 2019-08, Vol.19 (1), p.146-146, Article 146

2019

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Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis

The open respiratory medicine journal, 2016-12, Vol.10 (1), p.105-108

2016

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Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation

A small molecule CFTR potentiator restores ATP‐dependent channel gating to the cystic fibrosis mutant G551D‐CFTR

Modulation, microbiota and inflammation in the adult CF gut: A prospective study

Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non- G551D gating mutation

CORK Study in Cystic Fibrosis

Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation

CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor

Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR

GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1)

IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation

Ivacaftor partially corrects airway inflammation in a humanized G551D rat

Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis

On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: A molecular dynamic study

SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR

Pregnancy among cystic fibrosis women in the era of CFTR modulators

Improvement of Hepatic Steatosis in Cystic Fibrosis With Ivacaftor Therapy

G551D mutation impairs PKA-dependent activation of CFTR channel that can be restored by novel GOF mutations

Ivacaftor: Five‐year outcomes in the West of Scotland cystic fibrosis population

G551D-CFTR needs more bound actin than wild-type CFTR to maintain its presence in plasma membranes

Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations

Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor

Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations

Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies: Evaluation of: Accurso FJ, Rowe SM, Clancy JP, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010;363(21):1991-2003

Patient-reported outcomes in patients with cystic fibrosis with a G551D mutation on ivacaftor treatment: results from a cross-sectional study

Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis

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