Is CFTR-delF508 Really Absent from the Apical Membrane of the Airway Epithelium? CFTR-delF508 Expression in the Lower Airway
2011

CFTR-delF508 Expression in the Lower Airway

Sample size: 12 publication Evidence: moderate

Author Information

Author(s): Borthwick Lee A., Botha Phil, Verdon Bernard, Brodlie Malcolm J., Gardner Aaron, Bourn David, Johnson Gail E., Gray Mike A., Fisher Andrew J.

Primary Institution: Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom

Hypothesis

Is CFTR-delF508 really absent from the apical membrane of the airway epithelium?

Conclusion

CFTR is expressed at the apical membrane in epithelial cells from the lower airway but at a significantly reduced level compared to non-CF cells.

Supporting Evidence

  • CFTR-delF508 mutation is the most common cause of cystic fibrosis.
  • Previous studies suggested CFTR is absent from the apical membrane in CF patients.
  • This study found no significant difference in the percentage of cells expressing CFTR between CF and non-CF cells.

Takeaway

This study looked at how a common mutation in cystic fibrosis affects a protein in the lungs. It found that while the protein is there, it's not as strong as in healthy lungs.

Methodology

Epithelia were sampled by bronchial brushing and stained for CFTR to assess localization and expression levels.

Potential Biases

Potential bias in the interpretation of immunostaining results.

Limitations

The study's findings may not be generalizable due to the small sample size and specific patient population.

Participant Demographics

12 lung transplant recipients (8 females, 4 males) aged between 79 and 357 days post-transplant.

Statistical Information

P-Value

0.04

Statistical Significance

p=0.04

Digital Object Identifier (DOI)

10.1371/journal.pone.0023226

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