The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model

Martina Gentzsch, Deborah M. Cholon, Nancy L. Quinney, Susan E. Boyles, Mary E.B. Martino, Carla M.P. Ribeiro

Source: Eur Respir J, 52 (6) 1801133; 10.1183/13993003.01133-2018
Journal Issue: December
Disease area: Airway diseases

Congress or journal article abstractFull text journal articlePDF journal article, handout or slides

Rating: 0
You must login to grade this presentation.

Share or cite this content

Citations should be made in the following way:
Martina Gentzsch, Deborah M. Cholon, Nancy L. Quinney, Susan E. Boyles, Mary E.B. Martino, Carla M.P. Ribeiro. The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model. Eur Respir J, 52 (6) 1801133; 10.1183/13993003.01133-2018

You must login to share this Presentation/Article on Twitter, Facebook, LinkedIn or by email.

Member's Comments

No comment yet.
You must Login to comment this presentation.


Related content which might interest you:
Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration
Source: Eur Respir J, 59 (2) 2100185; 10.1183/13993003.00185-2021
Year: 2022



Correction of CFTR function in intestinal organoids to guide treatment of cystic fibrosis
Source: Eur Respir J, 57 (1) 1902426; 10.1183/13993003.02426-2019
Year: 2021



The cystic fibrosis lung microenvironment alters antibiotic activity: causes and effects
Source: Eur Respir Rev, 30 (161) 210055; 10.1183/16000617.0055-2021
Year: 2021



The CFTR and EGFR relationship in airway vascular growth, and its importance in cystic fibrosis
Source: Eur Respir J 2013; 42: 1438-1440
Year: 2013


A mathematical model to understand the airway clearance in cystic fibrosis
Source: International Congress 2019 – New developments in sleep-disordered breathing, gas exchange and lung function in children
Year: 2019

Mechanisms and markers of airway inflammation in cystic fibrosis
Source: Eur Respir J 2002; 19: 333-340
Year: 2002



New insights into the pathophysiology of lung disease in cystic fibrosis patients
Source: ISSN=ISSN 1025-448x, ISBN=ISBN 1-904097-46-6, page=1
Year: 2006

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis
Source: Eur Respir Rev 2013; 22: 58-65
Year: 2013



Characterization of cystic fibrosis airway epithelium in vitro
Source: Annual Congress 2005 - Cystic fibrosis lung disease: pieces of the puzzle
Year: 2005


Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies
Source: Eur Respir J 2017; 49(1): 1600903; DOI: 10.1183/13993003.00903-2016
Year: 2017



Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles
Source: ERJ Open Res, 5 (2) 00082-2019; 10.1183/23120541.00082-2019
Year: 2019



Improving airway clearance in cystic fibrosis lung disease
Source: Eur Respir Monogr 2014; 64: 169-187
Year: 2014


Targeting CFTR as a therapy for cystic fibrosis
Source: Lung Science Conference 2010 - Biology of gene-environment interactions in the lung
Year: 2010

Pharmacological treatment of the biochemical defect in cystic fibrosis airways
Source: Eur Respir J 2001; 17: 1314-1321
Year: 2001



RhDNase after airway clearance therapy improves peripheral airway patency in children with cystic fibrosis
Source: Annual Congress 2006 - Cystic fibrosis lung disease: molecules images and drugs
Year: 2006


Long-term clearance from small airways in patients with cystic fibrosis
Source: Eur Respir J 2005; 25: 317-323
Year: 2005



Investigation of the status of cystic fibrosis airway epithelial cells
Source: Annual Congress 2008 - Multigenic expression analyses: a guide for diagnosis and therapy
Year: 2008


Targeting cystic fibrosis inflammation in the age of CFTR modulators: focus on macrophages
Source: Eur Respir J, 57 (6) 2003502; 10.1183/13993003.03502-2020
Year: 2021



Evolution of biomechanical changes in a murine model of bleomycin-induced lung fibrosis: inflammation versus fibrosis
Source: Eur Respir J 2006; 28: Suppl. 50, 396s
Year: 2006