CFTR's Role in Regulating Inflammation in Cystic Fibrosis
Author Information
Author(s): Vij Neeraj, Mazur Steven, Zeitlin Pamela L.
Primary Institution: The Johns Hopkins School of Medicine
Hypothesis
Functional CFTR on the cell surface and its localization to cholesterol-rich lipid rafts is required for controlling NFκB activity and downstream inflammatory signaling.
Conclusion
CFTR is a negative regulator of NFκB mediated innate immune response, and its localization to lipid rafts is crucial for controlling inflammation.
Supporting Evidence
- CFTR expression in HEK293 cells suppresses IL-8 and NFκB promoter activities.
- CFTR knock out mice showed decreased survival and increased inflammatory response compared to wild type.
- Methyl-β-cyclodextrin treatment induced IL-8 and NFκB reporter activities, confirming CFTR's regulatory role.
Takeaway
CFTR helps keep our lungs healthy by controlling inflammation, and when it doesn't work properly, it can lead to more inflammation and problems in cystic fibrosis.
Methodology
The study involved co-expressing wt-CFTR and IL-8 or NFκB promoters in HEK293 cells and analyzing the effects on IL-8 secretion and NFκB activity.
Statistical Information
P-Value
p<0.01
Statistical Significance
p<0.05
Digital Object Identifier (DOI)
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