(Q38134182)

29 August 2017

title

Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence (English)

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29 August 2017

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Kavitha Kotha

1

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29 August 2017

John P Clancy

series ordinal

2

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29 August 2017

publication date

3 September 2013

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Therapeutic advances in respiratory disease

29 August 2017

published in

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29 August 2017

volume

7

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29 August 2017

issue

5

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29 August 2017

page(s)

288-296

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Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

29 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Calcium and pH-dependent packing and release of the gel-forming MUC2 mucin

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

21 January 2018

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https://api.crossref.org/works/10.1177%2F1753465813502115

Highlights of a workshop to discuss targeting inflammation in cystic fibrosis

21 January 2018

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https://api.crossref.org/works/10.1177%2F1753465813502115

Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

21 January 2018

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https://api.crossref.org/works/10.1177%2F1753465813502115

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Mucus clearance and lung function in cystic fibrosis with hypertonic saline

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Sodium channels and cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Expression of the cystic fibrosis gene in adult human lung

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Cystic fibrosis therapeutics: the road ahead

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Rethinking cystic fibrosis pathology: the critical role of abnormal reduced glutathione (GSH) transport caused by CFTR mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Current understanding of the inflammatory process in cystic fibrosis: onset and etiology

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Nucleotide release provides a mechanism for airway surface liquid homeostasis

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Multi-Ion mechanism for ion permeation and block in the cystic fibrosis transmembrane conductance regulator chloride channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Coordinated clearance of periciliary liquid and mucus from airway surfaces.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

A novel host defense system of airways is defective in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

CFTR function and prospects for therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Identification of the cystic fibrosis gene: chromosome walking and jumping

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Sputum biomarkers of inflammation in cystic fibrosis lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

CFTR as a cAMP-dependent regulator of sodium channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Mechanism of direct bicarbonate transport by the CFTR anion channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Functional analysis of F508del CFTR in native human colon

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Submucosal glands and airway defense

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

Ivacaftor potentiation of multiple CFTR channels with gating mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F1753465813502115

21 January 2018

Identifiers

DOI

10.1177/1753465813502115

1 reference

29 August 2017

1 reference