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Gomes-Alves P.,Instituto Nacional Of Sade Dr Ricardo Jorge Insa | Penque D.,Instituto Nacional Of Sade Dr Ricardo Jorge Insa
Expert Review of Proteomics | Year: 2010

The achievement and maintenance of a protein native conformation is a very complex cellular process involving a multitude of key factors whose contribution to a successful folding remains to be elucidated. On top of this, it is known that correct folding is crucial for proteins to play their normal role and, consequently, for the maintenance of cellular homeostasis or proteostasis. If the folding process is affected, the protein is unable to achieve its native conformation, compromising its life and function, and a pathological condition may arise. Protein-misfolding diseases are characterized by either formation of protein aggregates that are toxic to the cell (gain-of-toxic-function diseases) or by an incorrect processing of proteins, which leads to a deficiency in protein activity (loss-of-function diseases). In this article we have focused on proteomics advances in the molecular knowledge of protein-misfolding diseases with direct impact on possible key players in F508del-CFTR processing and trafficking. © 2010 Expert Reviews Ltd. Source


Gomes-Alves P.,Instituto Nacional Of Sade Dr Ricardo Jorge Insa | Neves S.,Instituto Nacional Of Sade Dr Ricardo Jorge Insa | Penque D.,Instituto Nacional Of Sade Dr Ricardo Jorge Insa
Methods in Enzymology | Year: 2011

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 low-temperature 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. © 2011 Elsevier Inc. Source

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