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Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi

2013-07Journal article Restricted access
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dc.contributor.authorFarinha, Carlos
dc.contributor.authorMatos, Paulo
dc.contributor.authorAmaral, Margardia
dc.date.accessioned2014-03-12T17:58:59Z
dc.date.available2014-03-12T17:58:59Z
dc.date.issued2013-07
dc.description.abstractBiogenesis of cystic fibrosis transmembrane conductance regulator (CFTR) starts with its cotranslational insertion into the membrane of the endoplasmic reticulum (ER) and core glycosylation. These initial events are followed by a complex succession of steps with the main goal of checking the overall quality of CFTR conformation in order to promote its exit from the ER through the secretory pathway. Failure to pass the various checkpoints of the ER quality control targets the most frequent disease-causing mutant protein (F508del-CFTR) for premature degradation. For wild-type CFTR that exits the ER, trafficking through the Golgi is the major site for glycan processing, although nonconventional trafficking pathways have also been described for CFTR. Once CFTR is at the cell surface, its stability is also controlled by multiple protein interactors, including Rab proteins, Rho small GTPases, and PDZ proteins. These regulate not only anterograde trafficking to the cell surface, but also endocytosis and recycling, thus achieving fine and tight modulation of CFTR plasma membrane levels. Exciting recent data have related autophagy and epithelial differentiation to the regulation of CFTR trafficking. Herein, we review the various checkpoints of the complex quality control along the secretory trafficking pathway and the associated pathways that are starting to be explored for the benefit of cystic fibrosis patients.por
dc.identifier.citationFEBS J. 2013 Sep;280(18):4396-406. doi: 10.1111/febs.12392. Epub 2013 Jul 5por
dc.identifier.issn1742-464X
dc.identifier.otherdoi: 10.1111/febs.12392
dc.identifier.urihttp://hdl.handle.net/10400.18/2101
dc.language.isoengpor
dc.peerreviewedyespor
dc.publisherWiley/ Federation of European Biochemical Societiespor
dc.relation.publisherversionhttp://onlinelibrary.wiley.com/doi/10.1111/febs.12392/fullpor
dc.subjectVias de Transdução de Sinal e Patologias Associadaspor
dc.subjectFibrose Quísticapor
dc.subjectDoença Genéticapor
dc.subjectCFTRpor
dc.subjectTrafficpor
dc.subjectRab GTPasespor
dc.subjectRho GTPasespor
dc.subjectTraffickingpor
dc.subjectChaperonespor
dc.subjectCystic Fibrosis Transmembrane Conductance Regulatorpor
dc.subjectEndocytosispor
dc.subjectEndoplasmic Reticulumpor
dc.subjectEndoplasmic Reticulum Quality Controlpor
dc.subjectFoldingpor
dc.titleControl of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgipor
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage4406por
oaire.citation.startPage4396por
oaire.citation.titleFEBS Journalpor
oaire.citation.volume280(18)por
rcaap.rightsrestrictedAccesspor
rcaap.typearticlepor

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