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Open AccessArticle

BAMBI and CHGA in Prion Diseases: Neuropathological Assessment and Potential Role as Disease Biomarkers

1
Laboratorio de Genética Bioquímica (LAGENBIO), Universidad de Zaragoza, Instituto Agroalimentario de Aragón-IA2, Instituto de Investigación Sanitaria Aragón-IISA, 50013 Zaragoza, Spain
2
Centro de Encefalopatías y Enfermedades Transmisibles Emergentes (CEETE), Universidad de Zaragoza, Instituto Agroalimentario de Aragón-IA2, Instituto de Investigación Sanitaria Aragón-IISA, 50013 Zaragoza, Spain
3
Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Institute Carlos III, 28029 Madrid, Spain
4
Instituto de Investigación Biomédica de Bellvitge (IDIBELL), L’Hospitalet de Llobregat, 08908 Barcelona, Spain
5
Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical School, 37075 Göttingen, Germany
6
Department of Biological Sciences, Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2R3, Canada
*
Author to whom correspondence should be addressed.
Biomolecules 2020, 10(5), 706; https://doi.org/10.3390/biom10050706
Received: 25 March 2020 / Revised: 25 April 2020 / Accepted: 29 April 2020 / Published: 2 May 2020
(This article belongs to the Special Issue Prion Disease Biomarkers: Recent Advances)
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. View Full-Text
Keywords: prion disease; scrapie; Creutzfeldt-Jakob disease; sheep; transgenic mice; biomarkers; neuroinflammation; bone morphogenetic protein and activin membrane-bound inhibitor; chromogranin A prion disease; scrapie; Creutzfeldt-Jakob disease; sheep; transgenic mice; biomarkers; neuroinflammation; bone morphogenetic protein and activin membrane-bound inhibitor; chromogranin A
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López-Pérez, Ó.; Bernal-Martín, M.; Hernaiz, A.; Llorens, F.; Betancor, M.; Otero, A.; Toivonen, J.M.; Zaragoza, P.; Zerr, I.; Badiola, J.J.; Bolea, R.; Martín-Burriel, I. BAMBI and CHGA in Prion Diseases: Neuropathological Assessment and Potential Role as Disease Biomarkers. Biomolecules 2020, 10, 706.

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