Indeed, previous studies have demonstrated direct effects of CFTR potentiation with ivacaftor on neutrophils,27 28 suggesting a fundamental role for CFTR in a number of neutrophil functions, consistent with CFTR being expressed at a biologically significant level.29 Our observation that apoptosis is impaired in neutrophils from CFTR null piglets underlines the potential primary nature of delayed neutrophil apoptosis in CF. Here, CFTR is linked to cystic fibrosis.