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Differential overexpression of SERPINA3 in human prion diseases
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-infe...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5688139/ https://www.ncbi.nlm.nih.gov/pubmed/29142239 http://dx.doi.org/10.1038/s41598-017-15778-8 |
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author | Vanni, S. Moda, F. Zattoni, M. Bistaffa, E. De Cecco, E. Rossi, M. Giaccone, G. Tagliavini, F. Haïk, S. Deslys, J. P. Zanusso, G. Ironside, J. W. Ferrer, I. Kovacs, G. G. Legname, G. |
author_facet | Vanni, S. Moda, F. Zattoni, M. Bistaffa, E. De Cecco, E. Rossi, M. Giaccone, G. Tagliavini, F. Haïk, S. Deslys, J. P. Zanusso, G. Ironside, J. W. Ferrer, I. Kovacs, G. G. Legname, G. |
author_sort | Vanni, S. |
collection | PubMed |
description | 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–Sträussler–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. |
format | Online Article Text |
id | pubmed-5688139 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-56881392017-11-24 Differential overexpression of SERPINA3 in human prion diseases Vanni, S. Moda, F. Zattoni, M. Bistaffa, E. De Cecco, E. Rossi, M. Giaccone, G. Tagliavini, F. Haïk, S. Deslys, J. P. Zanusso, G. Ironside, J. W. Ferrer, I. Kovacs, G. G. Legname, G. Sci Rep Article 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–Sträussler–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. Nature Publishing Group UK 2017-11-15 /pmc/articles/PMC5688139/ /pubmed/29142239 http://dx.doi.org/10.1038/s41598-017-15778-8 Text en © The Author(s) 2017 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/. |
spellingShingle | Article Vanni, S. Moda, F. Zattoni, M. Bistaffa, E. De Cecco, E. Rossi, M. Giaccone, G. Tagliavini, F. Haïk, S. Deslys, J. P. Zanusso, G. Ironside, J. W. Ferrer, I. Kovacs, G. G. Legname, G. Differential overexpression of SERPINA3 in human prion diseases |
title | Differential overexpression of SERPINA3 in human prion diseases |
title_full | Differential overexpression of SERPINA3 in human prion diseases |
title_fullStr | Differential overexpression of SERPINA3 in human prion diseases |
title_full_unstemmed | Differential overexpression of SERPINA3 in human prion diseases |
title_short | Differential overexpression of SERPINA3 in human prion diseases |
title_sort | differential overexpression of serpina3 in human prion diseases |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5688139/ https://www.ncbi.nlm.nih.gov/pubmed/29142239 http://dx.doi.org/10.1038/s41598-017-15778-8 |
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