Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties

Atsushi Kobayashi, Yuichi Matsuura, Toru Iwaki, Yasushi Iwasaki, Mari Yoshida, Hitoshi Takahashi, Shigeo Murayama, Masaki Takao, Shinsuke Kato, Masahito Yamada, Shirou Mohri, Tetsuyuki Kitamoto

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

The genotype (methionine, M or valine, V) at polymorphic codon 129 of the PRNP gene and the type (1 or 2) of abnormal prion protein in the brain are the major determinants of the clinicopathological features of sporadic Creutzfeldt-Jakob disease (CJD), thus providing molecular basis for classification of sporadic CJD, that is, MM1, MM2, MV1, MV2, VV1 or VV2. In addition to these "pure" cases, "mixed" cases presenting mixed neuropathological and biochemical features have also been recognized. The most frequently observed mixed form is the co-occurrence of MM1 and MM2, namely MM1+2. However, it has remained elusive whether MM1+2 could be a causative origin of dura mater graft-associated CJD (dCJD), one of the largest subgroups of iatrogenic CJD. To test this possibility, we performed transmission experiments of MM1+2 prions and a systematic neuropathological examination of dCJD patients in the present study. The transmission properties of the MM1+2 prions were identical to those of MM1 prions because MM2 prions lacked transmissibility. In addition, the neuropathological characteristics of MM2 were totally absent in dCJD patients examined. These results suggest that MM1+2 can be a causative origin of dCJD and causes neuropathological phenotype similar to that of MM1.

Original languageEnglish
Pages (from-to)95-101
Number of pages7
JournalBrain Pathology
Volume26
Issue number1
DOIs
Publication statusPublished - Jan 1 2016

Fingerprint

Prions
Creutzfeldt-Jakob Syndrome
Dura Mater
Valine
Codon
Methionine
Genotype
Transplants
Phenotype
Sporadic Creutzfeldt-Jakob Disease
Brain
Genes

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Neuroscience(all)
  • Clinical Neurology

Cite this

Kobayashi, A., Matsuura, Y., Iwaki, T., Iwasaki, Y., Yoshida, M., Takahashi, H., ... Kitamoto, T. (2016). Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties. Brain Pathology, 26(1), 95-101. https://doi.org/10.1111/bpa.12264

Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties. / Kobayashi, Atsushi; Matsuura, Yuichi; Iwaki, Toru; Iwasaki, Yasushi; Yoshida, Mari; Takahashi, Hitoshi; Murayama, Shigeo; Takao, Masaki; Kato, Shinsuke; Yamada, Masahito; Mohri, Shirou; Kitamoto, Tetsuyuki.

In: Brain Pathology, Vol. 26, No. 1, 01.01.2016, p. 95-101.

Research output: Contribution to journalArticle

Kobayashi, A, Matsuura, Y, Iwaki, T, Iwasaki, Y, Yoshida, M, Takahashi, H, Murayama, S, Takao, M, Kato, S, Yamada, M, Mohri, S & Kitamoto, T 2016, 'Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties', Brain Pathology, vol. 26, no. 1, pp. 95-101. https://doi.org/10.1111/bpa.12264
Kobayashi, Atsushi ; Matsuura, Yuichi ; Iwaki, Toru ; Iwasaki, Yasushi ; Yoshida, Mari ; Takahashi, Hitoshi ; Murayama, Shigeo ; Takao, Masaki ; Kato, Shinsuke ; Yamada, Masahito ; Mohri, Shirou ; Kitamoto, Tetsuyuki. / Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties. In: Brain Pathology. 2016 ; Vol. 26, No. 1. pp. 95-101.
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