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

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

Abstract 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 (resPrPD...

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Bibliographic Details
Main Authors: Laura Cracco, Xiangzhu Xiao, Satish K. Nemani, Jody Lavrich, Ignazio Cali, Bernardino Ghetti, Silvio Notari, Witold K. Surewicz, Pierluigi Gambetti
Format: Article
Language:English
Published: BMC 2019-05-01
Series:Acta Neuropathologica Communications
Subjects:
Creutzfeldt-Jakob disease
Prion protein
Aggregate formation
Multimers
Mass spectrometry
Epitope mapping
Online Access:http://link.springer.com/article/10.1186/s40478-019-0734-2

Internet

http://link.springer.com/article/10.1186/s40478-019-0734-2

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