A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy.

@article{Pohl2014ANI,
  title={A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy.},
  author={Kerstin Pohl and Elaine Mo Hayes and Joanne Keenan and Michael Henry and Paula Meleady and Kevin P. Molloy and Bakr Jundi and David A. Bergin and Cormac McCarthy and Oliver J McElvaney and Michelle M White and Martin M. Clynes and Emer P Reeves and Noel Gerry McElvaney},
  journal={Blood},
  year={2014},
  volume={124 7},
  pages={999-1009}
}
Studies have endeavored to reconcile whether dysfunction of neutrophils in people with cystic fibrosis (CF) is a result of the genetic defect or is secondary due to infection and inflammation. In this study, we illustrate that disrupted function of the CF transmembrane conductance regulator (CFTR), such as that which occurs in patients with ∆F508 and/or G551D mutations, correlates with impaired degranulation of antimicrobial proteins. We demonstrate that CF blood neutrophils release less… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 27 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 80 references

Attenuation of host defense function of lung BLOOD, 14 AUGUST 2014 x VOLUME 124, NUMBER 7 CFTR POTENTIATOR THERAPY AND NEUTROPHIL ACTIVITY

  • NE Alexis, MS Muhlebach, DB Peden, TL. Noah
  • For personal use only. on September
  • 2016

Similar Papers

Loading similar papers…