TY - JOUR
T1 - Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases
AU - Munesue, Yoshiko
AU - Shimazaki, Taishi
AU - Qi, Zechen
AU - Isoda, Norikazu
AU - Sawa, Hirofumi
AU - Aoshima, Keisuke
AU - Kimura, Takashi
AU - Mohri, Shirou
AU - Kitamoto, Tetsuyuki
AU - Kobayashi, Atsushi
N1 - Funding Information:
We thank H. Kudo, M. Yamamoto, and A. Yamazaki for their excellent technical assistance. This study was supported by Grants-in-Aid for Scientific Research from JSPS (A. Kobayashi), Research Grant from Japan Intractable Diseases Research Foundation (A. Kobayashi), and a Grant-in-Aid for Scientific Research on Innovative Areas (Brain Protein Aging and Dementia Control) from MEXT (T. Kitamoto).
Publisher Copyright:
© 2018
PY - 2018/3/6
Y1 - 2018/3/6
N2 - Evaluation of transmission properties is important for the differential diagnosis of a subgroup of acquired Creutzfeldt-Jakob disease (CJD) with methionine homozygosity at polymorphic codon 129 of the PRNP gene, an intermediate type abnormal prion protein (PrP), and kuru plaques, denoted as acquired CJD-MMiK. The present study aimed to develop a quick evaluation system of the transmission properties of acquired CJD-MMiK. In the PrP-humanized mice intraperitoneally inoculated with brain homogenates from an acquired CJD-MMiK patient, accumulation of abnormal PrP was observed in follicular dendritic cells of the spleen at 75 days post-inoculation. The transmission properties of acquired CJD-MMiK were quite different from those of sporadic CJD with the same PRNP codon 129 genotype. Moreover, even at 14 days post-inoculation, the characteristic transmission properties of acquired CJD-MMiK could be detected. These findings suggest that the bioassay using follicular dendritic cells of the spleen, named as a FDC assay, can be an easy, time-saving, and useful method to distinguish acquired CJD-MMiK from sporadic CJD.
AB - Evaluation of transmission properties is important for the differential diagnosis of a subgroup of acquired Creutzfeldt-Jakob disease (CJD) with methionine homozygosity at polymorphic codon 129 of the PRNP gene, an intermediate type abnormal prion protein (PrP), and kuru plaques, denoted as acquired CJD-MMiK. The present study aimed to develop a quick evaluation system of the transmission properties of acquired CJD-MMiK. In the PrP-humanized mice intraperitoneally inoculated with brain homogenates from an acquired CJD-MMiK patient, accumulation of abnormal PrP was observed in follicular dendritic cells of the spleen at 75 days post-inoculation. The transmission properties of acquired CJD-MMiK were quite different from those of sporadic CJD with the same PRNP codon 129 genotype. Moreover, even at 14 days post-inoculation, the characteristic transmission properties of acquired CJD-MMiK could be detected. These findings suggest that the bioassay using follicular dendritic cells of the spleen, named as a FDC assay, can be an easy, time-saving, and useful method to distinguish acquired CJD-MMiK from sporadic CJD.
KW - Creutzfeldt-Jakob disease
KW - Follicular dendritic cell
KW - Knock-in mouse
KW - Prion
KW - Transmissible spongiform encephalopathy
KW - Transmission property
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U2 - 10.1016/j.neulet.2018.01.014
DO - 10.1016/j.neulet.2018.01.014
M3 - Article
C2 - 29329906
AN - SCOPUS:85040247995
VL - 668
SP - 43
EP - 47
JO - Neuroscience Letters
JF - Neuroscience Letters
SN - 0304-3940
ER -