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Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases

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Title: Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases
Authors: Kobayashi, Atsushi Browse this author
Parchi, Piero Browse this author
Yamada, Masahito Browse this author →KAKEN DB
Mohri, Shirou Browse this author →KAKEN DB
Kitamoto, Tetsuyuki Browse this author →KAKEN DB
Keywords: Creutzfeldt-Jakob disease
iatrogenic
prion diseases
prions
PRNP
Issue Date: Jun-2016
Publisher: Wiley-Blackwell
Journal Title: Neuropathology
Volume: 36
Issue: 3
Start Page: 305
End Page: 310
Publisher DOI: 10.1111/neup.12270
PMID: 26669818
Abstract: As an experimental model of acquired Creutzfeldt-Jakob disease (CJD), we performed transmission studies of sporadic CJD using knock-in mice expressing human prion protein (PrP). In this model, the inoculation of the sporadic CJD strain V2 into animals homozygous for methionine at polymorphic codon 129 (129M/M) of the PRNP gene produced quite distinctive neuropathological and biochemical features, that is, widespread kuru plaques and intermediate type abnormal PrP (PrPSc). Interestingly, this distinctive combination of molecular and pathological features has been, to date, observed in acquired CJD but not in sporadic CJD. Assuming that these distinctive phenotypic traits are specific for acquired CJD, we revisited the literature and found two cases showing widespread kuru plaques despite the 129M/M genotype, in a neurosurgeon and in a patient with a medical history of neurosurgery without dura mater grafting. By Western blot analysis of brain homogenates, we revealed the intermediate type of PrPSc in both cases. Furthermore, transmission properties of brain extracts from these two cases were indistinguishable from those of a subgroup of dura mater graft-associated iatrogenic CJD caused by infection with the sporadic CJD strain V2. These data strongly suggest that the two atypical CJD cases, previously thought to represent sporadic CJD, very likely acquired the disease through exposure to prion-contaminated brain tissues. Thus, we propose that the distinctive combination of 129M/M genotype, kuru plaques, and intermediate type PrPSc, represents a reliable criterion for the identification of acquired CJD cases among presumed sporadic cases.
Rights: This is the peer reviewed version of the following article: Kobayashi, A., Parchi, P., Yamada, M., Mohri, S., and Kitamoto, T. (2016) Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases. Neuropathology, 36: 305–310, which has been published in final form at http://doi.org/10.1111/neup.12270. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving.
Type: article (author version)
URI: http://hdl.handle.net/2115/65887
Appears in Collections:獣医学院・獣医学研究院 (Graduate School of Veterinary Medicine / Faculty of Veterinary Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 小林 篤史

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