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http://hdl.handle.net/2445/124035
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DC Field | Value | Language |
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dc.contributor.author | Vanni, Silvia | - |
dc.contributor.author | Moda, Fabio | - |
dc.contributor.author | Zattoni, Marco | - |
dc.contributor.author | Bistaffa, E. | - |
dc.contributor.author | Cecco, E. De | - |
dc.contributor.author | Rossi, Marcello | - |
dc.contributor.author | Giaccone, Giorgio | - |
dc.contributor.author | Tagliavini, Fabrizio | - |
dc.contributor.author | Haik, Stéphane | - |
dc.contributor.author | Deslys, Jean-Philippe | - |
dc.contributor.author | Zanusso, Gianluigi | - |
dc.contributor.author | Ironside, James W. | - |
dc.contributor.author | Ferrer, Isidro (Ferrer Abizanda) | - |
dc.contributor.author | Kovacs, Gabor G. | - |
dc.contributor.author | Legname, Giuseppe | - |
dc.date.accessioned | 2018-07-27T12:22:02Z | - |
dc.date.available | 2018-07-27T12:22:02Z | - |
dc.date.issued | 2017-11-15 | - |
dc.identifier.uri | http://hdl.handle.net/2445/124035 | - |
dc.description.abstract | Prion diseases are fatal neurodegenerative disorders with sporadic, genetic or acquired etiologies. The molecular alterations leading to the onset and the spreading of these diseases are still unknown. In a previous work we identified a five-gene signature able to distinguish intracranially BSE-infected macaques from healthy ones, with SERPINA3 showing the most prominent dysregulation. We analyzed 128 suitable frontal cortex samples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal familial insomnia (FFI) n = 9, Gerstmann-Straussler-Scheinker syndrome (GSS)) n = 4), patients with Alzheimer disease (AD, n = 14) and age-matched controls (n = 30). Real Time-quantitative PCR was performed for SERPINA3 transcript, and ACTB, RPL19, GAPDH and B2M were used as reference genes. We report SERPINA3 to be strongly up-regulated in the brain of all human prion diseases, with only a mild up-regulation in AD. We show that this striking up-regulation, both at the mRNA and at the protein level, is present in all types of human prion diseases analyzed, although to a different extent for each specific disorder. Our data suggest that SERPINA3 may be involved in the pathogenesis and the progression of prion diseases, representing a valid tool for distinguishing different forms of these disorders in humans. | - |
dc.format.extent | 13 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Nature Publishing Group | - |
dc.relation.isformatof | Reproducció del document publicat a: http://dx.doi.org/10.1038/s41598-017-15778-8 | - |
dc.relation.ispartof | Scientific Reports, 2017, num. 7 | - |
dc.relation.uri | http://dx.doi.org/10.1038/s41598-017-15778-8 | - |
dc.rights | cc by (c) Vanni et al., 2017 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es/ | - |
dc.source | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) | - |
dc.subject.classification | Malalties neurodegeneratives | - |
dc.subject.classification | Etiologia | - |
dc.subject.classification | Malalties per prions | - |
dc.subject.other | Neurodegenerative Diseases | - |
dc.subject.other | Etiology | - |
dc.subject.other | Prion diseases | - |
dc.title | Differential overexpression of SERPINA3 in human prion diseases | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.date.updated | 2018-07-24T11:55:52Z | - |
dc.relation.projectID | info:eu-repo/grantAgreement/EC/H2020/643417/EU//JPco-fuND | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 29142239 | - |
Appears in Collections: | Articles publicats en revistes (Patologia i Terapèutica Experimental) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) Publicacions de projectes de recerca finançats per la UE |
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VanniS.pdf | 2.03 MB | Adobe PDF | View/Open |
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