Importantly, miglustat or Genz-529648, at the concentrations used in this study, strongly inhibited only GBA2 activity (figure 3); in parallel, we demonstrated a reduction of P. aeruginosa stimulated IL-8 mRNA expression and protein release in CF bronchial cells when GBA2 expression (figure 5) and function (figures 1 and 7) were decreased. This evidence concerns the gene GBA2 and cystic fibrosis.