CFTR, Cell Junctions and the Cytoskeleton
The multi-organ disease cystic fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, a cAMP regulated chloride (Cl<sup>−</sup>) and bicarbonate (HCO<sub>3</sub><sup>−</sup>) ion channel expressed at th...
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MDPI AG
2022-02-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/23/5/2688 |
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| author | Ines Pankonien Margarida C. Quaresma Cláudia S. Rodrigues Margarida D. Amaral |
| author_facet | Ines Pankonien Margarida C. Quaresma Cláudia S. Rodrigues Margarida D. Amaral |
| author_sort | Ines Pankonien |
| collection | DOAJ |
| description | The multi-organ disease cystic fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, a cAMP regulated chloride (Cl<sup>−</sup>) and bicarbonate (HCO<sub>3</sub><sup>−</sup>) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protein results in decreased Cl<sup>−</sup> secretion and excessive sodium reabsorption in epithelial cells, which consequently leads to epithelial dehydration and the accumulation of thick mucus within the affected organs, such as the lungs, pancreas, gastrointestinal (GI) tract, reproductive system and sweat glands. However, CFTR has been implicated in other functions besides transporting ions across epithelia. The rising number of references concerning its association to actin cytoskeleton organization, epithelial cell junctions and extracellular matrix (ECM) proteins suggests a role in the formation and maintenance of epithelial apical basolateral polarity. This review will focus on recent literature (the last 10 years) substantiating the role of CFTR in cell junction formation and actin cytoskeleton organization with its connection to the ECM. |
| first_indexed | 2024-03-09T20:37:30Z |
| format | Article |
| id | doaj.art-fabce5bbafac46caa456966558e4e50f |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-09T20:37:30Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-fabce5bbafac46caa456966558e4e50f2023-11-23T23:08:03ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672022-02-01235268810.3390/ijms23052688CFTR, Cell Junctions and the CytoskeletonInes Pankonien0Margarida C. Quaresma1Cláudia S. Rodrigues2Margarida D. Amaral3BioISI—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, PortugalBioISI—Biosystems and Integrative Sciences Institute, Faculty of Sciences, University of Lisboa, 1749-016 Lisboa, PortugalThe multi-organ disease cystic fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, a cAMP regulated chloride (Cl<sup>−</sup>) and bicarbonate (HCO<sub>3</sub><sup>−</sup>) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protein results in decreased Cl<sup>−</sup> secretion and excessive sodium reabsorption in epithelial cells, which consequently leads to epithelial dehydration and the accumulation of thick mucus within the affected organs, such as the lungs, pancreas, gastrointestinal (GI) tract, reproductive system and sweat glands. However, CFTR has been implicated in other functions besides transporting ions across epithelia. The rising number of references concerning its association to actin cytoskeleton organization, epithelial cell junctions and extracellular matrix (ECM) proteins suggests a role in the formation and maintenance of epithelial apical basolateral polarity. This review will focus on recent literature (the last 10 years) substantiating the role of CFTR in cell junction formation and actin cytoskeleton organization with its connection to the ECM.https://www.mdpi.com/1422-0067/23/5/2688cystic fibrosiscell polarizationepithelial differentiationepithelial regenerationepithelial-mesenchymal transitionextracellular matrix |
| spellingShingle | Ines Pankonien Margarida C. Quaresma Cláudia S. Rodrigues Margarida D. Amaral CFTR, Cell Junctions and the Cytoskeleton International Journal of Molecular Sciences cystic fibrosis cell polarization epithelial differentiation epithelial regeneration epithelial-mesenchymal transition extracellular matrix |
| title | CFTR, Cell Junctions and the Cytoskeleton |
| title_full | CFTR, Cell Junctions and the Cytoskeleton |
| title_fullStr | CFTR, Cell Junctions and the Cytoskeleton |
| title_full_unstemmed | CFTR, Cell Junctions and the Cytoskeleton |
| title_short | CFTR, Cell Junctions and the Cytoskeleton |
| title_sort | cftr cell junctions and the cytoskeleton |
| topic | cystic fibrosis cell polarization epithelial differentiation epithelial regeneration epithelial-mesenchymal transition extracellular matrix |
| url | https://www.mdpi.com/1422-0067/23/5/2688 |
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