Increased NF-κB activity and decreased Wnt/β-catenin signaling mediate reduced osteoblast differentiation and function in ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) mice

Abstract

Background: We analyzed the mechanisms mediating osteoblast dysfunctions in cystic fibrosis. Results: Osteoblast differentiation and function are impaired in ΔF508-CFTR mice due to overactive NF-κB and reduced Wnt/β-catenin signaling. Correcting these pathways rescued the defective osteoblast functions. Conclusion: Osteoblast dysfunctions in ΔF508-CFTR mice result from altered NF-κB and Wnt/β-catenin signaling. Significance: Targeting the altered signaling pathways can restore osteoblast functions in cystic fibrosis.