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A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease

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Title: A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease
Authors: Kobayashi, Atsushi Browse this author
Iwasaki, Yasushi Browse this author
Takao, Masaki Browse this author
Saito, Yuko Browse this author
Iwaki, Toru Browse this author
Qi, Zechen Browse this author
Torimoto, Ryouta Browse this author
Shimazaki, Taishi Browse this author
Munesue, Yoshiko Browse this author
Isoda, Norikazu Browse this author
Sawa, Hirofumi Browse this author
Aoshima, Keisuke Browse this author
Kimura, Takashi Browse this author →KAKEN DB
Kondo, Hinako Browse this author
Mohri, Shirou Browse this author
Kitamoto, Tetsuyuki Browse this author
Issue Date: Jun-2019
Publisher: Elsevier
Journal Title: The American Journal of Pathology
Volume: 189
Issue: 6
Start Page: 1276
End Page: 1283
Publisher DOI: 10.1016/j.ajpath.2019.02.012
Abstract: Six subgroups of sporadic Creutzfeldt-Jakob disease have been identified by distinctive clinicopathologic features, genotype at polymorphic codon 129 [methionine (M)/valine (V)] of the PRNP gene, and type of abnormal prion proteins (type 1 or 2). In addition to the pure subgroups, mixed neuropathologic features and the coexistence of two types of abnormal prion proteins in the same patient also have been reported. Here, we found that a portion of the patients previously diagnosed as MM1 had neuropathologic characteristics of the MM2 thalamic form (ie, neuronal loss of the inferior olivary nucleus of the medulla). Furthermore, coexistence of biochemical features of the MM2 thalamic form also was confirmed in the identified cases. In addition, in transmission experiments using prion protein humanized mice, the brain material from the identified case showed weak infectivity and generated characteristic abnormal prion proteins in the inoculated mice resembling those after inoculation with brain material of MM2 thalamic form. Taken together, these results show that the co-occurrence of MM1 and MM2 thalamic form is a novel entity of sporadic Creutzfeldt-Jakob disease prion strain co-occurrence. The present study raises the possibility that the co-occurrence of the MM2 thalamic form might have been overlooked so far because of the scarcity of abnormal prion protein accumulation and restricted neuropathology.
Rights: © 2019, Elsevier. Licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
Type: article (author version)
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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