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

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) mo...

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Main Authors: Margarida D. Amaral, Margarida C. Quaresma, Ines Pankonien
Format: Article
Language:English
Published: MDPI AG 2020-04-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/21/9/3133
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author Margarida D. Amaral
Margarida C. Quaresma
Ines Pankonien
author_facet Margarida D. Amaral
Margarida C. Quaresma
Ines Pankonien
author_sort Margarida D. Amaral
collection DOAJ
description 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.
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spelling doaj.art-a9a7c7353da44332afa414ce348fcb192023-11-19T23:00:58ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-04-01219313310.3390/ijms21093133What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?Margarida D. Amaral0Margarida C. Quaresma1Ines Pankonien2BioISI—Biosystems and Integrative Sciences Institute, Faculty of Sciences, University of Lisboa, 1749-016 Lisboa, PortugalBioISI—Biosystems and Integrative Sciences Institute, Faculty of Sciences, University of Lisboa, 1749-016 Lisboa, PortugalBioISI—Biosystems and Integrative Sciences Institute, Faculty of Sciences, University of Lisboa, 1749-016 Lisboa, PortugalOne 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.https://www.mdpi.com/1422-0067/21/9/3133carcinogenesiscystic fibrosisepithelial–mesenchymal transition EMTepithelial differentiationepithelial regenerationwound healing
spellingShingle Margarida D. Amaral
Margarida C. Quaresma
Ines Pankonien
What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
International Journal of Molecular Sciences
carcinogenesis
cystic fibrosis
epithelial–mesenchymal transition EMT
epithelial differentiation
epithelial regeneration
wound healing
title What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
title_full What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
title_fullStr What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
title_full_unstemmed What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
title_short What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
title_sort what role does cftr play in development differentiation regeneration and cancer
topic carcinogenesis
cystic fibrosis
epithelial–mesenchymal transition EMT
epithelial differentiation
epithelial regeneration
wound healing
url https://www.mdpi.com/1422-0067/21/9/3133
work_keys_str_mv AT margaridadamaral whatroledoescftrplayindevelopmentdifferentiationregenerationandcancer
AT margaridacquaresma whatroledoescftrplayindevelopmentdifferentiationregenerationandcancer
AT inespankonien whatroledoescftrplayindevelopmentdifferentiationregenerationandcancer