70332 20200108100343.0 doi 10.1097/MD.0000000000010320 sideral 105916 ART-2018-105916 eng (orcid)0000-0002-2787-9671 Monzón, M. Universidad de Zaragoza Glial alterations in human prion diseases 2018 Access copy available to the general public Unrestricted Background: Neuroinflammation has recently been proposed to be a major component of neurodegenerative diseases. The aim of this study was to determine how the interaction between microglia and astroglia, which are the primary immune cell populations in the brain, and pathological prion protein (PrPsc) could influence the development and propagation of this neurodegenerative disease. Because a relevant role for glial response in prion disease has been clearly demonstrated in our previous studies using the natural animal model, a similar approach has been taken here using the natural human model. Methods: A morphological approach has been developed to analyze cerebellar samples from patients with Creutzfeldt-Jakob disease (CJD) in comparison with healthy control cases. Histopathological lesions were assessed, and PrPsc, glial fibrillary acidic protein (GFAP) and reactive microglia were immunolabelled by specific antibodies. Furthermore, co-location studies using confocal microscopy were performed to determine the possible relationships between both types of glial cells in all samples. Results: The results presented in this study support the involvement of both types of glial cells in CJD. Evidence of increased astrocyte and microglia reactivity can be observed in all CJD cases, and a close relationship between the types of glia is demonstrated by co-location studies. Conclusion: Proteinopathies such as Alzheimer, Parkinson, and Huntington diseases, where aberrant proteins spread throughout the brain during disease progression, may share a molecular basis and mechanisms of propagation. Therefore, studies elucidating the interaction between gliosis and prion propagation may be relevant to these other neurodegenerative diseases and may provide new targets for therapeutic intervention. info:eu-repo/semantics/openAccess by http://creativecommons.org/licenses/by/3.0/es/ 1.87 2018 MEDICINE, GENERAL & INTERNAL 69 / 159 = 0.434 2018 Q2 T2 0.19 2018 Medicine (miscellaneous) 2018 Q3 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion (orcid)0000-0002-3423-0846 Hernández, R.S. Garcés, M. Universidad de Zaragoza Sarasa, R. (orcid)0000-0002-7173-7216 Badiola, J.J. Universidad de Zaragoza 1003 443 Universidad de Zaragoza Dpto. Anatom.Histolog.Humanas Area Histología 1009 773 Universidad de Zaragoza Dpto. Patología Animal Área Sanidad Animal 1007 610 Universidad de Zaragoza Dpto. Medicina, Psiqu. y Derm. Area Medicina 97, 15 (2018), e0320 Medicine (Baltim.) Medicine 0025-7974 2883144 http://zaguan.unizar.es/record/70332/files/texto_completo.pdf Versión publicada 123224 http://zaguan.unizar.es/record/70332/files/texto_completo.jpg?subformat=icon icon Versión publicada oai:zaguan.unizar.es:70332 articulos driver 2020-01-08-09:28:52 ARTICLE