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Title: Signaling Pathways of Proteostasis Network Unraveled by Proteomic Approaches on the Understanding of Misfolded Protein Rescue
Author: Gomes-Alves, Patricia
Neves, Sofia
Penque, Deborah
Keywords: Fibrose quistica
Genómica Funcional e Estrutural
Issue Date: 2011
Publisher: Elsevier
Citation: Methods Enzymol. 2011;491:217-33
Abstract: Attempts to promote normal processing and function of F508del-CFTR, the most common mutant in cystic fibrosis (CF), have been described as potential therapeutic strategies in the management of this disease. Here we described the proteomic approaches, namely two-dimensional electrophoresis (2DE), mass spectrometry (MS), and bioinformatics tools used in our recent studies to gain insight into the proteins potentially involved in lowtemperature or mutagenic treatment-induced rescue process of F508del-CFTR. The proteins identified are part of the proteostasis network, such as the unfolded protein response (UPR) signaling pathways that may regulate the processing of CF transmembrane conductance regulator (CFTR) through the folding and trafficking progression. The complete characterization of these signaling pathways and their regulators in CF will certainly contribute to the development of novel therapeutic strategies against CF.
Peer review: yes
ISBN: 978-0-12-385928-0
ISSN: 0076-6879
Publisher Version:
Appears in Collections:DGH - Capítulos (ou partes) de livros

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