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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
Malalties per prions
Neurodegenerative diseases
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.
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It is part of: Scientific Reports, 2017, num. 7
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Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Patologia i Terapèutica Experimental)

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