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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-02-01
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| Series: | Biomolecules |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2218-273X/13/3/425 |
| _version_ | 1797613287845658624 |
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| author | Óscar Fonseca Maria Salomé Gomes Maria Adelina Amorim Ana Cordeiro Gomes |
| author_facet | Óscar Fonseca Maria Salomé Gomes Maria Adelina Amorim Ana Cordeiro Gomes |
| author_sort | Óscar Fonseca |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-11T06:52:46Z |
| format | Article |
| id | doaj.art-9e35189fbfe14dc6b3c76dcceb3cfd22 |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-11T06:52:46Z |
| publishDate | 2023-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-9e35189fbfe14dc6b3c76dcceb3cfd222023-11-17T09:51:06ZengMDPI AGBiomolecules2218-273X2023-02-0113342510.3390/biom13030425Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic InflammationÓscar Fonseca0Maria Salomé Gomes1Maria Adelina Amorim2Ana Cordeiro Gomes3i3S–Instituto de Investigação e Inovação em Saúde, Universidade do Porto, 4200-135 Porto, Portugali3S–Instituto de Investigação e Inovação em Saúde, Universidade do Porto, 4200-135 Porto, PortugalCHUSJ–Centro Hospital Universitário de São João, 4200-319 Porto, Portugali3S–Instituto de Investigação e Inovação em Saúde, Universidade do Porto, 4200-135 Porto, PortugalCystic 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.https://www.mdpi.com/2218-273X/13/3/425cystic fibrosis related bone diseaseosteoporosisCFTRbone |
| spellingShingle | Óscar Fonseca Maria Salomé Gomes Maria Adelina Amorim Ana Cordeiro Gomes Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation Biomolecules cystic fibrosis related bone disease osteoporosis CFTR bone |
| 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 | cystic fibrosis related bone disease osteoporosis CFTR bone |
| url | https://www.mdpi.com/2218-273X/13/3/425 |
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