The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease

2018 ◽  
Vol 22 (8) ◽  
pp. 687-701 ◽  
Author(s):  
Patrick J. Moore ◽  
Robert Tarran
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Sodium Channel ◽  
Therapeutic Target ◽  
Epithelial Sodium Channel ◽  
Cystic Fibrosis Lung ◽  
Cystic Fibrosis Lung Disease ◽  
Epithelial Sodium Channel Enac

scholarly journals CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease

2015 ◽  
Vol 46 (2) ◽  
pp. 395-404 ◽  
Author(s):  
Melanie Carevic ◽  
Anurag Singh ◽  
Nikolaus Rieber ◽  
Olaf Eickmeier ◽  
Matthias Griese ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Lung Disease ◽  
Immune Cells ◽  
Therapeutic Target ◽  
Cystic Fibrosis Lung ◽  
Fungal Colonisation ◽  
Cystic Fibrosis Lung Disease ◽  
Potential Biomarker ◽  
Healthy Control

Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4+ granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4+ airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease.

scholarly journals Risk Factors for the Progression of Cystic Fibrosis Lung Disease throughout Childhood

2014 ◽  
Vol 11 (1) ◽  
pp. 63-72 ◽  
Author(s):  
Don B. Sanders ◽  
Zhanhai Li ◽  
Anita Laxova ◽  
Michael J. Rock ◽  
Hara Levy ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
Cystic Fibrosis Lung Disease

scholarly journals Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease

Nature ◽  
2009 ◽  
Vol 458 (7241) ◽  
pp. 1039-1042 ◽  
Author(s):  
YuanYuan Gu ◽  
Isaac T. W. Harley ◽  
Lindsay B. Henderson ◽  
Bruce J. Aronow ◽  
Ilja Vietor ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Modifier Gene ◽  
Cystic Fibrosis Lung ◽  
Cystic Fibrosis Lung Disease
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