{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T00:44:46Z","timestamp":1760402686751,"version":"build-2065373602"},"reference-count":48,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2020,5,2]],"date-time":"2020-05-02T00:00:00Z","timestamp":1588377600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100010067","name":"Gobierno de Arag\u00f3n","doi-asserted-by":"publisher","award":["C012\/2014 and C020\/2014 and Order IIU\/2023\/2017","Grupo de Referencia LAGENBIO (A19-17R)"],"award-info":[{"award-number":["C012\/2014 and C020\/2014 and Order IIU\/2023\/2017","Grupo de Referencia LAGENBIO (A19-17R)"]}],"id":[{"id":"10.13039\/501100010067","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004895","name":"European Social Fund","doi-asserted-by":"publisher","award":["C012\/2014 and C020\/2014 and Order IIU\/2023\/2017","Construyendo Europa desde Arag\u00f3n: Grupo de Referencia LAGENBIO (A19-17R)"],"award-info":[{"award-number":["C012\/2014 and C020\/2014 and Order IIU\/2023\/2017","Construyendo Europa desde Arag\u00f3n: Grupo de Referencia LAGENBIO (A19-17R)"]}],"id":[{"id":"10.13039\/501100004895","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100010198","name":"Ministerio de Econom\u00eda, Industria y Competitividad, Gobierno de Espa\u00f1a","doi-asserted-by":"publisher","award":["AGL2015-67945-P"],"award-info":[{"award-number":["AGL2015-67945-P"]}],"id":[{"id":"10.13039\/501100010198","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004837","name":"Ministerio de Ciencia e Innovaci\u00f3n","doi-asserted-by":"publisher","award":["RTI2018-098711-B-I00"],"award-info":[{"award-number":["RTI2018-098711-B-I00"]}],"id":[{"id":"10.13039\/501100004837","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","award":["AGL2015-67945-P, RTI2018-098711-B-I00"],"award-info":[{"award-number":["AGL2015-67945-P, RTI2018-098711-B-I00"]}],"id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI19\/00144"],"award-info":[{"award-number":["PI19\/00144"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/biom10050706","type":"journal-article","created":{"date-parts":[[2020,5,5]],"date-time":"2020-05-05T06:41:20Z","timestamp":1588660880000},"page":"706","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["BAMBI and CHGA in Prion Diseases: Neuropathological Assessment and Potential Role as Disease Biomarkers"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2454-2114","authenticated-orcid":false,"given":"\u00d3scar","family":"L\u00f3pez-P\u00e9rez","sequence":"first","affiliation":[{"name":"Laboratorio de Gen\u00e9tica Bioqu\u00edmica (LAGENBIO), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"},{"name":"Centro de Encefalopat\u00edas y Enfermedades Transmisibles Emergentes (CEETE), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CIBERNED), Institute Carlos III, 28029 Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Biom\u00e9dica de Bellvitge (IDIBELL), L\u2019Hospitalet de Llobregat, 08908 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcos","family":"Bernal-Mart\u00edn","sequence":"additional","affiliation":[{"name":"Laboratorio de Gen\u00e9tica Bioqu\u00edmica (LAGENBIO), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6806-9990","authenticated-orcid":false,"given":"Adelaida","family":"Hernaiz","sequence":"additional","affiliation":[{"name":"Laboratorio de Gen\u00e9tica Bioqu\u00edmica (LAGENBIO), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9756-7497","authenticated-orcid":false,"given":"Franc","family":"Llorens","sequence":"additional","affiliation":[{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CIBERNED), Institute Carlos III, 28029 Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Biom\u00e9dica de Bellvitge (IDIBELL), L\u2019Hospitalet de Llobregat, 08908 Barcelona, Spain"},{"name":"Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical School, 37075 G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0388-9429","authenticated-orcid":false,"given":"Marina","family":"Betancor","sequence":"additional","affiliation":[{"name":"Centro de Encefalopat\u00edas y Enfermedades Transmisibles Emergentes (CEETE), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alicia","family":"Otero","sequence":"additional","affiliation":[{"name":"Centro de Encefalopat\u00edas y Enfermedades Transmisibles Emergentes (CEETE), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"},{"name":"Department of Biological Sciences, Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2R3, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7243-1737","authenticated-orcid":false,"given":"Janne Markus","family":"Toivonen","sequence":"additional","affiliation":[{"name":"Laboratorio de Gen\u00e9tica Bioqu\u00edmica (LAGENBIO), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 Zaragoza, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CIBERNED), Institute Carlos III, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pilar","family":"Zaragoza","sequence":"additional","affiliation":[{"name":"Laboratorio de Gen\u00e9tica Bioqu\u00edmica (LAGENBIO), Universidad de Zaragoza, Instituto Agroalimentario de Arag\u00f3n-IA2, Instituto de Investigaci\u00f3n Sanitaria Arag\u00f3n-IISA, 50013 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Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,5,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1126\/science.6801762","article-title":"Novel proteinaceous infectious particles cause scrapie","volume":"216","author":"Prusiner","year":"1982","journal-title":"Science"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1136\/vr.80.1.2","article-title":"The possible nature of the transmissible agent of scrapie","volume":"80","author":"Pattison","year":"1967","journal-title":"Veter. 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