Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.18/5500 |
Resumo: | The amount of ion channels and transporters present at the plasma membrane is a crucial component of the overall regulation of ion transport. The number of channels present result from an intricate network of proteins that controls the late events of channel trafficking, such as endocytosis, recycling and targeting to lysosomal degradation. Small GTPases of the Rab family are key players in these processes thus contributing to regulation of fluid secretion and ion homeostasis. In epithelia, this involves mainly the balance between the chloride channel CFTR and the sodium channel ENaC, whose misfunction is a hallmark of cystic fibrosis - the commonest recessive disorder in Caucasians. Here, we review the role of GTPases in regulating trafficking of ion channels and transporters, comparing what is known for CFTR and ENaC with other types of channels. We also discuss how feasible would be to target the Rab machinery to handle a disorder such as CF. |
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Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosisGTPaseCystic FibrosisMembrane TrafficVias de Transdução de Sinal e Patologias AssociadasThe amount of ion channels and transporters present at the plasma membrane is a crucial component of the overall regulation of ion transport. The number of channels present result from an intricate network of proteins that controls the late events of channel trafficking, such as endocytosis, recycling and targeting to lysosomal degradation. Small GTPases of the Rab family are key players in these processes thus contributing to regulation of fluid secretion and ion homeostasis. In epithelia, this involves mainly the balance between the chloride channel CFTR and the sodium channel ENaC, whose misfunction is a hallmark of cystic fibrosis - the commonest recessive disorder in Caucasians. Here, we review the role of GTPases in regulating trafficking of ion channels and transporters, comparing what is known for CFTR and ENaC with other types of channels. We also discuss how feasible would be to target the Rab machinery to handle a disorder such as CF.Work supported by center grant (to BioISI, Centre Reference: UID/MULTI/04046/2013), grants IF2012 (FCT/MCTES, Portugal) and PGG-055–2014 (Gilead Gen ese Portugal) to P.M. and grant PGG-039–2014 (Gilead Gen ese Portugal) and Romain Pauwels Research Award to C.M.F. The funders had no role in the preparation of the manuscript.Taylor & FrancisRepositório Científico do Instituto Nacional de SaúdeFarinha, CarlosMatos, Paulo2018-03-29T12:12:05Z2017-05-022017-05-02T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.18/5500engSmall GTPases. 2018 Mar 4;9(1-2):136-144. doi: 10.1080/21541248.2017.1317700. Epub 2017 May 19.2154-124810.1080/21541248.2017.1317700info:eu-repo/semantics/embargoedAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-07-20T15:40:53Zoai:repositorio.insa.pt:10400.18/5500Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T18:40:10.369559Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
title |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
spellingShingle |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis Farinha, Carlos GTPase Cystic Fibrosis Membrane Traffic Vias de Transdução de Sinal e Patologias Associadas |
title_short |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
title_full |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
title_fullStr |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
title_full_unstemmed |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
title_sort |
Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis |
author |
Farinha, Carlos |
author_facet |
Farinha, Carlos Matos, Paulo |
author_role |
author |
author2 |
Matos, Paulo |
author2_role |
author |
dc.contributor.none.fl_str_mv |
Repositório Científico do Instituto Nacional de Saúde |
dc.contributor.author.fl_str_mv |
Farinha, Carlos Matos, Paulo |
dc.subject.por.fl_str_mv |
GTPase Cystic Fibrosis Membrane Traffic Vias de Transdução de Sinal e Patologias Associadas |
topic |
GTPase Cystic Fibrosis Membrane Traffic Vias de Transdução de Sinal e Patologias Associadas |
description |
The amount of ion channels and transporters present at the plasma membrane is a crucial component of the overall regulation of ion transport. The number of channels present result from an intricate network of proteins that controls the late events of channel trafficking, such as endocytosis, recycling and targeting to lysosomal degradation. Small GTPases of the Rab family are key players in these processes thus contributing to regulation of fluid secretion and ion homeostasis. In epithelia, this involves mainly the balance between the chloride channel CFTR and the sodium channel ENaC, whose misfunction is a hallmark of cystic fibrosis - the commonest recessive disorder in Caucasians. Here, we review the role of GTPases in regulating trafficking of ion channels and transporters, comparing what is known for CFTR and ENaC with other types of channels. We also discuss how feasible would be to target the Rab machinery to handle a disorder such as CF. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-05-02 2017-05-02T00:00:00Z 2018-03-29T12:12:05Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10400.18/5500 |
url |
http://hdl.handle.net/10400.18/5500 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Small GTPases. 2018 Mar 4;9(1-2):136-144. doi: 10.1080/21541248.2017.1317700. Epub 2017 May 19. 2154-1248 10.1080/21541248.2017.1317700 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/embargoedAccess |
eu_rights_str_mv |
embargoedAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Taylor & Francis |
publisher.none.fl_str_mv |
Taylor & Francis |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799132143265251328 |