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Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments

Despite their phenotypic heterogeneity, most human prion diseases belong to two broadly defined groups: Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS). While the structural characteristics of the disease-related proteinase K-resistant prion protein (resPrP(D)) assoc...

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Detalles Bibliográficos
Autores principales: Cracco, Laura, Xiao, Xiangzhu, Nemani, Satish K., Lavrich, Jody, Cali, Ignazio, Ghetti, Bernardino, Notari, Silvio, Surewicz, Witold K., Gambetti, Pierluigi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6540574/
https://www.ncbi.nlm.nih.gov/pubmed/31142381
http://dx.doi.org/10.1186/s40478-019-0734-2