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Signaling Pathways of Proteostasis Network Unraveled by Proteomic Approaches on the Understanding of Misfolded Protein Rescue
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Orientador(es)
Resumo(s)
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.
Descrição
Palavras-chave
Fibrose quistica Proteómica Genómica Funcional e Estrutural
Contexto Educativo
Citação
Methods Enzymol. 2011;491:217-33