Abstract
In this issue of Blood, Pohl et al demonstrate that neutrophils from patients with mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) have impaired exocytosis of gelatinase and specific granules, altered ion homeostasis resulting in deactivation of the small GTPase Rab27a, and compromise bacterial killing abilities. These neutrophil defects were corrected by ivacaftor treatment, an ion channel potentiator, which has shown beneficial effects in pulmonary function in cystic fibrosis (CF) patients.