The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
In cystic fibrosis (CF), p.Phe508del is the most frequent mutation in the Cystic Fibrosis Transmembrane conductance Regulator (<i>CFTR</i>) gene. The p.Phe508del-CFTR protein is retained in the ER and rapidly degraded. This retention likely triggers an atypical Unfolded Protein Response...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-11-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/10/11/2980 |
| _version_ | 1827677002250846208 |
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| author | Pascal Trouvé Claude Férec Emmanuelle Génin |
| author_facet | Pascal Trouvé Claude Férec Emmanuelle Génin |
| author_sort | Pascal Trouvé |
| collection | DOAJ |
| description | In cystic fibrosis (CF), p.Phe508del is the most frequent mutation in the Cystic Fibrosis Transmembrane conductance Regulator (<i>CFTR</i>) gene. The p.Phe508del-CFTR protein is retained in the ER and rapidly degraded. This retention likely triggers an atypical Unfolded Protein Response (UPR) involving ATF6, which reduces the expression of p.Phe508del-CFTR. There are still some debates on the role of the UPR in CF: could it be triggered by the accumulation of misfolded CFTR proteins in the endoplasmic reticulum as was proposed for the most common CFTR mutation p.Phe508del? Or, is it the consequence of inflammation and infection that occur in the disease? In this review, we summarize recent findings on UPR in CF and show how infection, inflammation and UPR act together in CF. We propose to rethink their respective role in CF and to consider them as a whole. |
| first_indexed | 2024-03-10T05:37:09Z |
| format | Article |
| id | doaj.art-30f8e4ee39904309b41976d16eda6351 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T05:37:09Z |
| publishDate | 2021-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-30f8e4ee39904309b41976d16eda63512023-11-22T22:49:35ZengMDPI AGCells2073-44092021-11-011011298010.3390/cells10112980The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic FibrosisPascal Trouvé0Claude Férec1Emmanuelle Génin2Inserm, Univ Brest, EFS, UMR 1078, GGB, F-29200 Brest, FranceInserm, Univ Brest, EFS, UMR 1078, GGB, F-29200 Brest, FranceInserm, Univ Brest, EFS, UMR 1078, GGB, F-29200 Brest, FranceIn cystic fibrosis (CF), p.Phe508del is the most frequent mutation in the Cystic Fibrosis Transmembrane conductance Regulator (<i>CFTR</i>) gene. The p.Phe508del-CFTR protein is retained in the ER and rapidly degraded. This retention likely triggers an atypical Unfolded Protein Response (UPR) involving ATF6, which reduces the expression of p.Phe508del-CFTR. There are still some debates on the role of the UPR in CF: could it be triggered by the accumulation of misfolded CFTR proteins in the endoplasmic reticulum as was proposed for the most common CFTR mutation p.Phe508del? Or, is it the consequence of inflammation and infection that occur in the disease? In this review, we summarize recent findings on UPR in CF and show how infection, inflammation and UPR act together in CF. We propose to rethink their respective role in CF and to consider them as a whole.https://www.mdpi.com/2073-4409/10/11/2980cystic fibrosisunfolded protein responseinflammationinfection |
| spellingShingle | Pascal Trouvé Claude Férec Emmanuelle Génin The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis Cells cystic fibrosis unfolded protein response inflammation infection |
| title | The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis |
| title_full | The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis |
| title_fullStr | The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis |
| title_full_unstemmed | The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis |
| title_short | The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis |
| title_sort | interplay between the unfolded protein response inflammation and infection in cystic fibrosis |
| topic | cystic fibrosis unfolded protein response inflammation infection |
| url | https://www.mdpi.com/2073-4409/10/11/2980 |
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