The Possible Pathogenesis of Idiopathic Pulmonary Fibrosis considering MUC5B.

BACKGROUND

Overexpression of the MUC5B protein is associated with idiopathic pulmonary fibrosis (IPF), but little information is available regarding the pathogenic effects and regulatory mechanisms of overexpressed MUC5B in IPF.

MAIN BODY: The overexpression of MUC5B in terminal bronchi and honeycomb cysts produces mucosal host defensive dysfunction in the distal airway which may play an important role in the development of IPF. This review addresses the possible association of overexpression of MUC5B, with MUC5B promoter polymorphism, MUC5B gene epigenetic changes, effects of some transcriptional factors, and inflammatory mediators in IPF. In addition, the associated signaling pathways which may influence the expression of MUC5B are also discussed.

CONCLUSION

This work has important implications for further exploration of the mechanisms of overexpression of MUC5B in IPF, and future personalized treatment.