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.
| Original language | English |
|---|---|
| Pages (from-to) | 1276-1283 |
| Number of pages | 8 |
| Journal | American Journal of Pathology |
| Volume | 189 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Jun 1 2019 |
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All Science Journal Classification (ASJC) codes
- Pathology and Forensic Medicine
Cite this
A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease. / Kobayashi, Atsushi; Iwasaki, Yasushi; Takao, Masaki; Saito, Yuko; Iwaki, Toru; Qi, Zechen; Torimoto, Ryouta; Shimazaki, Taishi; Munesue, Yoshiko; Isoda, Norikazu; Sawa, Hirofumi; Aoshima, Keisuke; Kimura, Takashi; Kondo, Hinako; Mohri, Shirou; Kitamoto, Tetsuyuki.
In: American Journal of Pathology, Vol. 189, No. 6, 01.06.2019, p. 1276-1283.Research output: Contribution to journal › Article
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TY - JOUR
T1 - A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease
AU - Kobayashi, Atsushi
AU - Iwasaki, Yasushi
AU - Takao, Masaki
AU - Saito, Yuko
AU - Iwaki, Toru
AU - Qi, Zechen
AU - Torimoto, Ryouta
AU - Shimazaki, Taishi
AU - Munesue, Yoshiko
AU - Isoda, Norikazu
AU - Sawa, Hirofumi
AU - Aoshima, Keisuke
AU - Kimura, Takashi
AU - Kondo, Hinako
AU - Mohri, Shirou
AU - Kitamoto, Tetsuyuki
PY - 2019/6/1
Y1 - 2019/6/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85065614688&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85065614688&partnerID=8YFLogxK
U2 - 10.1016/j.ajpath.2019.02.012
DO - 10.1016/j.ajpath.2019.02.012
M3 - Article
VL - 189
SP - 1276
EP - 1283
JO - American Journal of Pathology
JF - American Journal of Pathology
SN - 0002-9440
IS - 6
ER -