(Q42973495)

English

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

scientific article published on 9 July 2010

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scholarly article

1 reference

Europe PubMed Central

12 November 2017

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

1 reference

Europe PubMed Central

12 November 2017

transmembrane protein

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cystic fibrosis

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5

object named as

Teresinha Leal

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Europe PubMed Central

12 November 2017

Francois Vermeulen

4

object named as

François Vermeulen

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Europe PubMed Central

12 November 2017

author name string

Isabelle Sermet-Gaudelus

1

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Europe PubMed Central

12 November 2017

Kris De Boeck

2

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Europe PubMed Central

12 November 2017

Georges J Casimir

3

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Europe PubMed Central

12 November 2017

Agnès Mogenet

6

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Europe PubMed Central

12 November 2017

Delphine Roussel

7

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Europe PubMed Central

12 November 2017

Janine Fritsch

8

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Europe PubMed Central

12 November 2017

Laurence Hanssens

9

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Europe PubMed Central

12 November 2017

Samit Hirawat

10

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Europe PubMed Central

12 November 2017

Nilsen L Miller

11

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Europe PubMed Central

12 November 2017

Scott Constantine

12

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Europe PubMed Central

12 November 2017

Allen Reha

13

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Europe PubMed Central

12 November 2017

Temitayo Ajayi

14

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Europe PubMed Central

12 November 2017

Gary L Elfring

15

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Europe PubMed Central

12 November 2017

Langdon L Miller

16

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Europe PubMed Central

12 November 2017

language of work or name

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publication date

9 July 2010

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12 November 2017

American Journal of Respiratory and Critical Care Medicine

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12 November 2017

182

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12 November 2017

10

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1262-1272

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12 November 2017

Cystic fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis

1 reference

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Detecting early structural lung damage in cystic fibrosis.

1 reference

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High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

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Jointly modelling the relationship between survival and pulmonary function in cystic fibrosis patients.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

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Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial

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Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group

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21 January 2018

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

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The changing face of the exocrine pancreas in cystic fibrosis: pancreatic sufficiency, pancreatitis and genotype.

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https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Three, four or more: the translational stop signal at length

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Genotype-phenotype correlation for pulmonary function in cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

CFTR Genotype as a Predictor of Prognosis in Cystic Fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis andCFTRStop Mutations

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

PTC124 targets genetic disorders caused by nonsense mutations

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Nasal potential difference measurements in patients with atypical cystic fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Basic protocol for transepithelial nasal potential difference measurements

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Chloride transport in nasal ciliated cells of cystic fibrosis heterozygotes.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Pulmonary function between 6 and 18 years of age.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

High treatment burden in adults with cystic fibrosis: challenges to disease self-management

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Early intervention and prevention of lung disease in cystic fibrosis: a European consensus

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

CFTR delivery to 25% of surface epithelial cells restores normal rates of mucus transport to human cystic fibrosis airway epithelium

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Molecular mechanisms of beta(2)-adrenergic receptor function, response, and regulation

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Impact of different chloride and glucose solutions on nasal potential difference.

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Assessment of the CFTR and ENaC association

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201001-0137OC

21 January 2018

Identifiers

10.1164/RCCM.201001-0137OC

1 reference

Europe PubMed Central

12 November 2017

1 reference

Europe PubMed Central

12 November 2017

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