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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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Autores principales: Amaral, Margarida D., Quaresma, Margarida C., Pankonien, Ines
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7246864/
https://www.ncbi.nlm.nih.gov/pubmed/32365523
http://dx.doi.org/10.3390/ijms21093133
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author Amaral, Margarida D.
Quaresma, Margarida C.
Pankonien, Ines
author_facet Amaral, Margarida D.
Quaresma, Margarida C.
Pankonien, Ines
author_sort Amaral, Margarida D.
collection PubMed
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 pubmed-72468642020-06-02 What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer? Amaral, Margarida D. Quaresma, Margarida C. Pankonien, Ines Int J Mol Sci Review 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. MDPI 2020-04-29 /pmc/articles/PMC7246864/ /pubmed/32365523 http://dx.doi.org/10.3390/ijms21093133 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Amaral, Margarida D.
Quaresma, Margarida C.
Pankonien, Ines
What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
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 Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7246864/
https://www.ncbi.nlm.nih.gov/pubmed/32365523
http://dx.doi.org/10.3390/ijms21093133
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