Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.18/2101
Título: Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi
Autor: Farinha, Carlos
Matos, Paulo
Amaral, Margardia
Palavras-chave: Vias de Transdução de Sinal e Patologias Associadas
Fibrose Quística
Doença Genética
CFTR
Traffic
Rab GTPases
Rho GTPases
Trafficking
Chaperones
Cystic Fibrosis Transmembrane Conductance Regulator
Endocytosis
Endoplasmic Reticulum
Endoplasmic Reticulum Quality Control
Folding
Data: Jul-2013
Editora: Wiley/ Federation of European Biochemical Societies
Citação: FEBS J. 2013 Sep;280(18):4396-406. doi: 10.1111/febs.12392. Epub 2013 Jul 5
Resumo: Biogenesis 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.
Peer review: yes
URI: http://hdl.handle.net/10400.18/2101
ISSN: 1742-464X
Versão do Editor: http://onlinelibrary.wiley.com/doi/10.1111/febs.12392/full
Aparece nas colecções:DGH - Artigos em revistas internacionais

Ficheiros deste registo:
Ficheiro Descrição TamanhoFormato 
Rev CFTR traffic Paulo_MDA FEBS J 13.pdf424,38 kBAdobe PDFVer/Abrir    Acesso Restrito. Solicitar cópia ao autor!


FacebookTwitterDeliciousLinkedInDiggGoogle BookmarksMySpace
Formato BibTex MendeleyEndnote Degois 

Todos os registos no repositório estão protegidos por leis de copyright, com todos os direitos reservados.