Cystic fibrosis transmembrane conductance regulator dysfunction and its treatment (Q26851242)

language of work or name

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June 2012

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Medico-Chirurgical Transactions

published in

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volume

105 Suppl 2

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page(s)

S2-8

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

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

7 April 2017

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Sodium channel blockers for cystic fibrosis

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

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Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

7 April 2017

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

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

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Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.

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Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function

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Sodium channels and cystic fibrosis

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Gene transfer of CFTR to airway epithelia: low levels of expression are sufficient to correct Cl- transport and overexpression can generate basolateral CFTR.

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Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

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Relative ion permeability of normal and cystic fibrosis nasal epithelium

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Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin

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Short-term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis.

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Duramycin enhances chloride secretion in airway epithelium

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Stimulation of sodium transport by duramycin in cultured human colonic epithelia

31 May 2018

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Ion channel formation by duramycin

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A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis

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Pharmacological Properties of N-(3,5-Diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine Methanesulfonate (552-02), a Novel Epithelial Sodium Channel Blocker with Potential Clinical Efficacy for Cystic Fibrosis L

21 January 2018

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Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease

21 January 2018

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A phase I trial of intranasal Moli1901 for cystic fibrosis

21 January 2018

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Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

21 January 2018

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Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis

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Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis

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French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group

21 January 2018

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12 December 2020

Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses

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12 December 2020

No added benefit from nebulized amiloride in patients with cystic fibrosis

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12 December 2020

Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function

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12 December 2020

Mechanisms and applications of hypertonic saline.

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12 December 2020

Inhaled dry powder mannitol in cystic fibrosis: an efficacy and safety study

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12 December 2020

A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis

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12 December 2020

Identifiers

DOI

10.1258/JRSM.2012.12S001

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PubMed publication ID

22688363

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