In agreement with results obtained in cells cultured directly from uninfected CF tissues, Weber et al., [38] have found that defects of CFTR, in function of localization, contribute to endogenous activation of NF-κB, and consequently to the exaggerated production of the pro-inflammatory cytokine IL-8, even in the absence of bacteria. Here, NFKB1 is linked to cystic fibrosis.