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Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation
Cystic fibrosis is a monogenic disease with a multisystemic phenotype, ranging from predisposition to chronic lung infection and inflammation to reduced bone mass. The exact mechanisms unbalancing the maintenance of an optimal bone mass in cystic fibrosis patients remain unknown. Multiple factors ma...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10046889/ https://www.ncbi.nlm.nih.gov/pubmed/36979360 http://dx.doi.org/10.3390/biom13030425 |
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author | Fonseca, Óscar Gomes, Maria Salomé Amorim, Maria Adelina Gomes, Ana Cordeiro |
author_facet | Fonseca, Óscar Gomes, Maria Salomé Amorim, Maria Adelina Gomes, Ana Cordeiro |
author_sort | Fonseca, Óscar |
collection | PubMed |
description | Cystic fibrosis is a monogenic disease with a multisystemic phenotype, ranging from predisposition to chronic lung infection and inflammation to reduced bone mass. The exact mechanisms unbalancing the maintenance of an optimal bone mass in cystic fibrosis patients remain unknown. Multiple factors may contribute to severe bone mass reduction that, in turn, have devastating consequences in the patients’ quality of life and longevity. Here, we will review the existing evidence linking the CFTR dysfunction and cell-intrinsic bone defects. Additionally, we will also address how the proinflammatory environment due to CFTR dysfunction in immune cells and chronic infection impairs the maintenance of an adequate bone mass in CF patients. |
format | Online Article Text |
id | pubmed-10046889 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-100468892023-03-29 Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation Fonseca, Óscar Gomes, Maria Salomé Amorim, Maria Adelina Gomes, Ana Cordeiro Biomolecules Review Cystic fibrosis is a monogenic disease with a multisystemic phenotype, ranging from predisposition to chronic lung infection and inflammation to reduced bone mass. The exact mechanisms unbalancing the maintenance of an optimal bone mass in cystic fibrosis patients remain unknown. Multiple factors may contribute to severe bone mass reduction that, in turn, have devastating consequences in the patients’ quality of life and longevity. Here, we will review the existing evidence linking the CFTR dysfunction and cell-intrinsic bone defects. Additionally, we will also address how the proinflammatory environment due to CFTR dysfunction in immune cells and chronic infection impairs the maintenance of an adequate bone mass in CF patients. MDPI 2023-02-24 /pmc/articles/PMC10046889/ /pubmed/36979360 http://dx.doi.org/10.3390/biom13030425 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Fonseca, Óscar Gomes, Maria Salomé Amorim, Maria Adelina Gomes, Ana Cordeiro Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation |
title | Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation |
title_full | Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation |
title_fullStr | Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation |
title_full_unstemmed | Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation |
title_short | Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation |
title_sort | cystic fibrosis bone disease: the interplay between cftr dysfunction and chronic inflammation |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10046889/ https://www.ncbi.nlm.nih.gov/pubmed/36979360 http://dx.doi.org/10.3390/biom13030425 |
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