Please use this identifier to cite or link to this item:
http://hdl.handle.net/2445/173565
Title: | BAMBI and CHGA in Prion Diseases: Neuropathological Assessment and Potential Role as Disease Biomarkers |
Author: | López Pérez, Óscar Bernal Martín, Marcos Hernaiz, Adelaida Llorens Torres, Franc Betancor, Marina Otero, Alicia Toivonen, Janne Markus Zaragoza, Pilar Zerr, Inga Badiola, Juan José Bolea, Rosa Martín Burriel, Inmaculada |
Keywords: | Malalties per prions Malaltia de Creutzfeldt-Jakob Prion diseases Creutzfeldt-Jakob disease |
Issue Date: | 1-May-2020 |
Publisher: | MDPI |
Abstract: | Prion diseases affect both animals and humans. Research in the natural animal model of the disease could help in the understanding of neuropathological mechanisms and in the development of biomarkers for human pathologies. For this purpose, we studied the expression of 10 genes involved in prion propagation in vitro in the central nervous system of scrapie-infected sheep. Dysregulated genes (BAMBI and CHGA) were further analysed in a transgenic murine model (Tg338) of scrapie, and their protein distribution was determined using immunohistochemistry and Western blot. Their potential as biomarkers was finally assessed using enzyme-linked immunosorbent assay (ELISA) in cerebrospinal fluid (CSF) of scrapie sheep and Creutzfeldt-Jakob disease (CJD) patients. Protein BAMBI was upregulated in highly affected brain areas and CHGA was overexpressed along the brain in both models. Moreover, BAMBI and CHGA immunostaining scores strongly correlated with spongiosis and microgliosis in mice. Finally, levels of BAMBI were significantly higher in the CSF of clinical sheep and CJD patients. In addition to their potential as biomarkers, our work confirms the role of BAMBI and CHGA in prion neuropathology in vivo, but besides prion replication, they seem to be involved in the characteristic neuroinflammatory response associated to prion infection. |
Note: | Reproducció del document publicat a: https://doi.org/10.3390/biom10050706 |
It is part of: | Biomolecules, 2020, vol. 10, num. 5 |
URI: | http://hdl.handle.net/2445/173565 |
Related resource: | https://doi.org/10.3390/biom10050706 |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
Files in This Item:
File | Description | Size | Format | |
---|---|---|---|---|
Lopez-PerezO.pdf | 9.29 MB | Adobe PDF | View/Open |
This item is licensed under a Creative Commons License