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What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?

Authors :
Margarida D. Amaral
Margarida C. Quaresma
Ines Pankonien
Source :
International Journal of Molecular Sciences, Vol 21, Iss 3133, p 3133 (2020), International Journal of Molecular Sciences
Publication Year :
2020
Publisher :
MDPI AG, 2020.

Abstract

One of the key features associated with the substantial increase in life expectancy for individuals with CF is an elevated predisposition to cancer, firmly established by recent studies involving large cohorts. With the recent advances in cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies and the increased long-term survival rate of individuals with cystic fibrosis (CF), this is a novel challenge emerging at the forefront of this disease. However, the mechanisms linking dysfunctional CFTR to carcinogenesis have yet to be unravelled. Clues to this challenging open question emerge from key findings in an increasing number of studies showing that CFTR plays a role in fundamental cellular processes such as foetal development, epithelial differentiation/polarization, and regeneration, as well as in epithelial–mesenchymal transition (EMT). Here, we provide state-of-the-art descriptions on the moonlight roles of CFTR in these processes, highlighting how they can contribute to novel therapeutic strategies. However, such roles are still largely unknown, so we need rapid progress in the elucidation of the underlying mechanisms to find the answers and thus tailor the most appropriate therapeutic approaches.

Details

Language :
English
ISSN :
16616596 and 14220067
Volume :
21
Issue :
3133
Database :
OpenAIRE
Journal :
International Journal of Molecular Sciences
Accession number :
edsair.doi.dedup.....c1d5e6b14bbaec51f31b66601f38b4a0