Synthetic Aminoglycosides Efficiently Suppress Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations and Are Enhanced by Ivacaftor

Nonsense Mutations ◽

WS01.3 Translational read-through of CFTR nonsense mutations and inducement of cystic fibrosis transmembrane conductance regulator (CFTR) function by ELX-02 treatment

Journal of Cystic Fibrosis ◽

10.1016/s1569-1993(18)30121-8 ◽

2018 ◽

Vol 17 ◽

pp. S2 ◽

Cited By ~ 3

Author(s):

S.M. Rowe ◽

V. Mutyam ◽

I. Alroy ◽

P. Huertas

Keyword(s):

Cystic Fibrosis ◽

Nonsense Mutations ◽

Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis.

Journal of Clinical Investigation ◽

10.1172/jci115510 ◽

1991 ◽

Vol 88 (6) ◽

pp. 1880-1885 ◽

Cited By ~ 72

Author(s):

A Hamosh ◽

B C Trapnell ◽

P L Zeitlin ◽

C Montrose-Rafizadeh ◽

B J Rosenstein ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Messenger Rna ◽

Respiratory Epithelial Cells ◽

Nonsense Mutations ◽

Severe Deficiency ◽

Respiratory Epithelial ◽

Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations

American Journal of Respiratory and Critical Care Medicine ◽

10.1164/rccm.201601-0154oc ◽

2016 ◽

Vol 194 (9) ◽

pp. 1092-1103 ◽

Cited By ~ 55

Author(s):

Venkateshwar Mutyam ◽

Ming Du ◽

Xiaojiao Xue ◽

Kim M. Keeling ◽

E. Lucile White ◽

...

Keyword(s):

Cystic Fibrosis ◽

Nonsense Mutations ◽

Der Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) und Sphingolipide regulieren die hypoxisch pulmonale Vasokonstriktion

Pneumologie ◽

10.1055/s-0036-1571988 ◽

2016 ◽

Vol 70 (S 01) ◽

Author(s):

C Tabeling ◽

H Yu ◽

L Wang ◽

H Ranke ◽

NM Goldenberg ◽

...

Keyword(s):

Cystic Fibrosis ◽

Faculty Opinions recommendation of Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1024340.288770 ◽

2005 ◽

Author(s):

William H Colledge

Keyword(s):

Cystic Fibrosis ◽

Channel Activity ◽

Cl Channel ◽

Faculty Opinions recommendation of Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1046329.496274 ◽

2006 ◽

Author(s):

Davis Ng

Keyword(s):

Cystic Fibrosis ◽

Quality Control ◽

Faculty Opinions recommendation of Resistance to Pseudomonas aeruginosa chronic lung infection requires cystic fibrosis transmembrane conductance regulator-modulated interleukin-1 (IL-1) release and signaling through the IL-1 receptor.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1065903.518850 ◽

2007 ◽

Author(s):

Joanna Goldberg

Keyword(s):

Cystic Fibrosis ◽

Pseudomonas Aeruginosa ◽

Interleukin 1 ◽

Lung Infection ◽

Cystic Fibrosis Transmembrane ◽

Chronic Lung Infection

Faculty Opinions recommendation of Identification of new small molecule inhibitors of cystic fibrosis transmembrane conductance regulator protein: in vitro and in vivo studies.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1069767.522696 ◽

2007 ◽

Author(s):

Kim Barrett

Keyword(s):

Cystic Fibrosis ◽

Small Molecule ◽

Small Molecule Inhibitors ◽

In Vivo Studies ◽

Regulator Protein ◽

Faculty Opinions recommendation of MicroRNA regulation of expression of the cystic fibrosis transmembrane conductance regulator gene.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.13361055.14731200 ◽

2011 ◽

Author(s):

Pradeep Dudeja ◽

Arivarasu Natarajan Anbazhagan

Keyword(s):

Cystic Fibrosis ◽

Regulator Gene ◽

Regulation Of Expression ◽

Microrna Regulation ◽

Faculty Opinions recommendation of Cystic fibrosis transmembrane conductance regulator is expressed in mucin granules from Calu-3 and primary human airway epithelial cells.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718017856.793479176 ◽

2013 ◽

Author(s):

Steven M Rowe ◽

Sammeta Vamsee Raju

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Airway Epithelial ◽

Human Airway ◽

Cystic Fibrosis Transmembrane ◽

Human Airway Epithelial Cells