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 ) and bicarbonate (HCO ) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protei...
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| Veröffentlicht in: | International journal of molecular sciences 2022-02, Vol.23 (5), p.2688 |
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| creator | Pankonien, Ines Quaresma, Margarida C Rodrigues, Cláudia S Amaral, Margarida D |
| 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
) and bicarbonate (HCO
) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protein results in decreased Cl
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. |
| doi_str_mv | 10.3390/ijms23052688 |
| format | Article |
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) and bicarbonate (HCO
) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protein results in decreased Cl
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.</description><identifier>ISSN: 1422-0067</identifier><identifier>ISSN: 1661-6596</identifier><identifier>EISSN: 1422-0067</identifier><identifier>DOI: 10.3390/ijms23052688</identifier><identifier>PMID: 35269829</identifier><language>eng</language><publisher>Switzerland: MDPI AG</publisher><subject>Actin ; Bicarbonates ; Bicarbonates - metabolism ; Cell junctions ; Chlorides - metabolism ; Cystic fibrosis ; Cystic Fibrosis - genetics ; Cystic Fibrosis - metabolism ; Cystic fibrosis transmembrane conductance regulator ; Cystic Fibrosis Transmembrane Conductance Regulator - genetics ; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism ; Cytoskeleton ; Cytoskeleton - metabolism ; Dehydration ; Epithelial cells ; Epithelial Cells - metabolism ; Epithelium ; Extracellular matrix ; Humans ; Intercellular Junctions - metabolism ; Kinases ; Mutation ; Organs ; Pancreas ; Polarity ; Proteins ; Reabsorption ; Reproductive system ; Review ; Signal transduction ; Sweat gland</subject><ispartof>International journal of molecular sciences, 2022-02, Vol.23 (5), p.2688</ispartof><rights>2022 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 (https://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2022 by the authors. 2022</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c412t-9702c9e3bd39592d2e242b96f2f4006f707fccafdf771e6f2f6aa5c205fc2f933</citedby><cites>FETCH-LOGICAL-c412t-9702c9e3bd39592d2e242b96f2f4006f707fccafdf771e6f2f6aa5c205fc2f933</cites><orcidid>0000-0002-4622-7521 ; 0000-0001-8771-4820 ; 0000-0001-7978-4685 ; 0000-0002-0828-8630</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8910340/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8910340/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,881,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/35269829$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Pankonien, Ines</creatorcontrib><creatorcontrib>Quaresma, Margarida C</creatorcontrib><creatorcontrib>Rodrigues, Cláudia S</creatorcontrib><creatorcontrib>Amaral, Margarida D</creatorcontrib><title>CFTR, Cell Junctions and the Cytoskeleton</title><title>International journal of molecular sciences</title><addtitle>Int J Mol Sci</addtitle><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
) and bicarbonate (HCO
) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protein results in decreased Cl
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.</description><subject>Actin</subject><subject>Bicarbonates</subject><subject>Bicarbonates - metabolism</subject><subject>Cell junctions</subject><subject>Chlorides - metabolism</subject><subject>Cystic fibrosis</subject><subject>Cystic Fibrosis - genetics</subject><subject>Cystic Fibrosis - metabolism</subject><subject>Cystic fibrosis transmembrane conductance regulator</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - metabolism</subject><subject>Cytoskeleton</subject><subject>Cytoskeleton - metabolism</subject><subject>Dehydration</subject><subject>Epithelial cells</subject><subject>Epithelial Cells - metabolism</subject><subject>Epithelium</subject><subject>Extracellular matrix</subject><subject>Humans</subject><subject>Intercellular Junctions - metabolism</subject><subject>Kinases</subject><subject>Mutation</subject><subject>Organs</subject><subject>Pancreas</subject><subject>Polarity</subject><subject>Proteins</subject><subject>Reabsorption</subject><subject>Reproductive system</subject><subject>Review</subject><subject>Signal transduction</subject><subject>Sweat gland</subject><issn>1422-0067</issn><issn>1661-6596</issn><issn>1422-0067</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>BENPR</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNpdkd9LwzAQx4Mobk7ffJaCLwqrJpe2aV4EKc4fDASZzyFLE9fZJrNphf33tmyO6dMddx--d987hM4JvqGU49tiWXmgOIYkTQ_QkEQAIcYJO9zLB-jE-yXGQCHmx2hAO5qnwIfoOpvM3sZBpssyeGmtagpnfSBtHjQLHWTrxvlPXerG2VN0ZGTp9dk2jtD75GGWPYXT18fn7H4aqohAE3KGQXFN5znlMYccNEQw54kBE3WrGIaZUUqa3DBGdF9OpIwV4NgoMJzSEbrb6K7aeaVzpW1Ty1Ks6qKS9Vo4WYi_HVssxIf7FiknmEa4E7jaCtTuq9W-EVXhVWdQWu1aLyChKSMQ037W5T906dradvZ6irGIxiTpqPGGUrXzvtZmtwzBov-B2P9Bh1_sG9jBv0enP02sgRI</recordid><startdate>20220228</startdate><enddate>20220228</enddate><creator>Pankonien, Ines</creator><creator>Quaresma, Margarida C</creator><creator>Rodrigues, Cláudia S</creator><creator>Amaral, Margarida D</creator><general>MDPI AG</general><general>MDPI</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>8G5</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2O</scope><scope>MBDVC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0002-4622-7521</orcidid><orcidid>https://orcid.org/0000-0001-8771-4820</orcidid><orcidid>https://orcid.org/0000-0001-7978-4685</orcidid><orcidid>https://orcid.org/0000-0002-0828-8630</orcidid></search><sort><creationdate>20220228</creationdate><title>CFTR, Cell Junctions and the Cytoskeleton</title><author>Pankonien, Ines ; 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) and bicarbonate (HCO
) ion channel expressed at the apical plasma membrane (PM) of epithelial cells. Reduced CFTR protein results in decreased Cl
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.</abstract><cop>Switzerland</cop><pub>MDPI AG</pub><pmid>35269829</pmid><doi>10.3390/ijms23052688</doi><orcidid>https://orcid.org/0000-0002-4622-7521</orcidid><orcidid>https://orcid.org/0000-0001-8771-4820</orcidid><orcidid>https://orcid.org/0000-0001-7978-4685</orcidid><orcidid>https://orcid.org/0000-0002-0828-8630</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Actin Bicarbonates Bicarbonates - metabolism Cell junctions Chlorides - metabolism Cystic fibrosis Cystic Fibrosis - genetics Cystic Fibrosis - metabolism Cystic fibrosis transmembrane conductance regulator Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Cytoskeleton Cytoskeleton - metabolism Dehydration Epithelial cells Epithelial Cells - metabolism Epithelium Extracellular matrix Humans Intercellular Junctions - metabolism Kinases Mutation Organs Pancreas Polarity Proteins Reabsorption Reproductive system Review Signal transduction Sweat gland |
| title | CFTR, Cell Junctions and the Cytoskeleton |
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