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Title: | Differential overexpression of SERPINA3 in human prion diseases |
Author: | Vanni, Silvia Moda, Fabio Zattoni, Marco Bistaffa, E. Cecco, E. De Rossi, Marcello Giaccone, Giorgio Tagliavini, Fabrizio Haik, Stéphane Deslys, Jean-Philippe Zanusso, Gianluigi Ironside, James W. Ferrer, Isidro (Ferrer Abizanda) Kovacs, Gabor G. Legname, Giuseppe |
Keywords: | Malalties neurodegeneratives Etiologia Malalties per prions Neurodegenerative Diseases Etiology Prion diseases |
Issue Date: | 15-Nov-2017 |
Publisher: | Nature Publishing Group |
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. |
Note: | Reproducció del document publicat a: http://dx.doi.org/10.1038/s41598-017-15778-8 |
It is part of: | Scientific Reports, 2017, num. 7 |
URI: | http://hdl.handle.net/2445/124035 |
Related resource: | http://dx.doi.org/10.1038/s41598-017-15778-8 |
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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