title: Signaling pathways of proteostasis network unraveled by proteomic approaches on the understanding of misfolded protein rescue.
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 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.
authors.affiliation: Laboratório de Proteómica, Departamento de Genética, Instituto Nacional de Saúde Dr Ricardo Jorge (INSA, I.P.), Av. Padre Cruz, Lisboa, Portugal.
authors.forename: Patrícia
authors.initials: P
authors.lastname: Gomes-Alves
authors.forename: Sofia
authors.initials: S
authors.lastname: Neves
authors.forename: Deborah
authors.initials: D
authors.lastname: Penque
mesh_terms: D000818:Animals; D002460:Cell Line; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism; D015180:Electrophoresis, Gel, Two-Dimensional / Q000379:methods; D006801:Humans; D011506:Proteins / Q000235:genetics / Q000378:metabolism; D040901:Proteomics / Q000379:methods*; D015398:Signal Transduction; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization / Q000379:methods; D056811:Unfolded Protein Response
chemical_list: D011506:Proteins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator