Atypical Creutzfeldt-Jakob disease with PrP-amyloid plaques in white matter: molecular characterization and transmission to bank voles show the M1 strain signature

Abstract Amyloid plaques formed by abnormal prion protein (PrPSc) aggregates occur with low frequency in Creutzfeldt-Jakob disease, but represent a pathological hallmark of three relatively rare disease histotypes, namely variant CJD, sporadic CJDMV2K (methionine/valine at PRNP codon 129, PrPSc type...

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Bibliographic Details
Main Authors: Marcello Rossi, Daniela Saverioni, Michele Di Bari, Simone Baiardi, Afina Willemina Lemstra, Laura Pirisinu, Sabina Capellari, Annemieke Rozemuller, Romolo Nonno, Piero Parchi
Format: Article
Language:English
Published: BMC 2017-11-01
Series:Acta Neuropathologica Communications
Subjects:
CJD
Online Access:http://link.springer.com/article/10.1186/s40478-017-0496-7