(Q46082134)

19 December 2017

title

Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice (English)

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19 December 2017

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7

Teresinha Leal

1 reference

19 December 2017

Rita Vanbever

4

object named as

Rita Vanbever

1 reference

19 December 2017

author name string

Bob Lubamba

1

1 reference

19 December 2017

Jean Lebacq

2

1 reference

19 December 2017

Patrick Lebecque

3

1 reference

19 December 2017

Anissa Leonard

5

1 reference

19 December 2017

Pierre Wallemacq

6

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19 December 2017

language of work or name

English

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publication date

19 March 2009

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19 December 2017

American Journal of Respiratory and Critical Care Medicine

published in

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19 December 2017

volume

179

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19 December 2017

issue

11

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19 December 2017

page(s)

1022-1028

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19 December 2017

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

1 reference

Cystic fibrosis since 1938.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

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/10.1164%2FRCCM.200901-0049OC

Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

What have we learned from mouse models for cystic fibrosis?

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Sustained therapeutic effects of oral miglustat (Zavesca, N-butyldeoxynojirimycin, OGT 918) in type I Gaucher disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Antiviral effect of alpha-glucosidase inhibitors on viral morphogenesis and binding properties of hepatitis C virus-like particles.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Anti-inflammatory effect of miglustat in bronchial epithelial cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

A cystic fibrosis respiratory epithelial cell chronically treated by miglustat acquires a non-cystic fibrosis-like phenotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Successful protocol of anaesthesia for measuring transepithelial nasal potential difference in spontaneously breathing mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Nasal application of the cationic liposome DC-Chol:DOPE does not alter ion transport, lung function or bacterial growth

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Generation and characterization of a delta F508 cystic fibrosis mouse model.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

A mouse model for the delta F508 allele of cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Inhalation therapy: technological milestones in asthma treatment

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Coupling modes and stoichiometry of Cl-/HCO3- exchange by slc26a3 and slc26a6

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Role of calnexin in the ER quality control and productive folding of CFTR; differential effect of calnexin knockout on wild-type and DeltaF508 CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Development of chronic bronchitis and emphysema in beta-epithelial Na+ channel-overexpressing mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

Fenretinide corrects newly found ceramide deficiency in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

The delivery site of a monovalent influenza vaccine within the respiratory tract impacts on the immune response

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200901-0049OC

10.1164/RCCM.200901-0049OC

21 January 2018

Identifiers

DOI

1 reference

19 December 2017

PubMed ID

19299496

1 reference

19 December 2017