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Prolonged survival of neutrophils from patients with ΔF508 CFTR mutations

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Cystic fibrosis (CF) is an autosomal recessive “channelopathy” characterised by aberrant CFTR and ENaC function resulting in widespread epithelial cell dysfunction and persistent airway infection. Studies indicating that airway inflammation precedes infection1 and that patients with CF display exaggerated neutrophilic responses to pathogens have suggested a primary defect in innate immune responses in CF. Given the importance of apoptosis to the resolution of neutrophilic inflammation,2 we sought to determine whether circulating CF neutrophils display normal apoptotic capacity.

Peripheral blood neutrophils were isolated from 12 clinically stable Pseudomonas colonised ΔF508 homozygote adult patients with CF and 12 age and sex matched healthy controls using discontinuous plasma/Percoll gradients.3 Blood neutrophil counts were in the normal range …

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  • Funding: Funded by the CF Trust UK (DJM), Wellcome Trust (AMC), Asthma-UK (ASC, NF), Papworth Hospital and BLF (KCC).

  • Competing interests: None.

  • Ethics approval: Ethics approval was obtained.

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