Journal
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
Volume 9, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fcell.2021.611921
Keywords
cystic fibrosis; iPSCs; bone differentiation; osteoblast; osteoclast
Categories
Funding
- French association Vaincre la Mucoviscidose [RF20200502686/1/1/132]
- Region Grand Est
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Cystic fibrosis-related bone disease is characterized by decreased bone formation and increased bone resorption, impacted by CFTR mutations that affect osteoblast and osteoclast differentiation and disrupt signaling pathways involved in stem cell commitment to bone lineages. The role of CFTR in inflammation and Wnt signaling in osteoblasts, as well as its potential direct impact on osteoclast function, is also highlighted.
Cystic fibrosis (CF)-related bone disease has emerged as a significant comorbidity of CF and is characterized by decreased bone formation and increased bone resorption. Both osteoblast and osteoclast differentiations are impacted by cystic fibrosis transmembrane conductance regulator (CFTR) mutations. The defect of CFTR chloride channel or the loss of CFTR's ability to interact with other proteins affect several signaling pathways involved in stem cell differentiation and the commitment of these cells toward bone lineages. Specifically, TGF-beta, nuclear factor-kappa B (NF-kappa B), PI3K/AKT, and MAPK/ERK signaling are disturbed by CFTR mutations, thus perturbing stem cell differentiation. High inflammation in patients changes myeloid lineage secretion, affecting both myeloid and mesenchymal differentiation. In osteoblast, Wnt signaling is impacted, resulting in consequences for both bone formation and resorption. Finally, CFTR could also have a direct role in osteoclast's resorptive function. In this review, we summarize the existing literature on the role of CFTR mutations on the commitment of induced pluripotent stem cells to bone cells.
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