A striking long-term consequence of this NEDD4-2 deficiency in murine airways, however, was the development of patchy lung fibrosis, bronchiolar remodelling, and increased MUC5B production in the peripheral airways, all features strongly reminiscent of IPF and actually reflecting IPF pathology more accurately than the most commonly used bleomycin-induced mouse model of lung fibrosis [58]. Here, MUC5B is linked to pulmonary fibrosis.