Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases

Munesue, Yoshiko; Shimazaki, Taishi; Qi, Zechen; Isoda, Norikazu; Sawa, Hirofumi; Aoshima, Keisuke; Kimura, Takashi; Mohri, Satoshi; Kitamoto, Tetsuyuki; Kobayashi, Atsushi

doi:10.1016/j.neulet.2018.01.014

Cited by 5 publications

(9 citation statements)

References 32 publications

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“…The V2 strain-inoculated Ki-Hu129M/M mice produced the intermediate type PrP Sc (20 kDa), while the V2 strain-inoculated Ki-Hu129V/V mice produced type 2 PrP Sc (19 kDa) (Fig. 1), as reported previously [15,20]. In contrast, the M1 sCJD strain was not efficiently transmitted to the human PrP C knock-in mice by the peripheral route.…”

Section: Resultssupporting

confidence: 82%

“…The inoculated mice were sacrificed at 75 days post-inoculation, and the spleen was immediately frozen for western blotting. This bioassay system has been developed to evaluate rapidly the infectivity of PrP Sc through the peripheral route [15,17,18].…”

Section: Transmission Experimentsmentioning

confidence: 99%

“…The reason for the different proportions of the causative sCJD strains among acquired prion diseases has remained elusive. Recently, we established a quick bioassay to evaluate the infectivity of PrP Sc by intraperitoneal inoculation and found that a M1 sCJD isolate did not infect efficiently through the peripheral route [15]. This finding has raised the possibility that the predominance of the V2 strain subgroup in human growth hormone-associated iCJD and kuru might have been due to the weak infectivity of the M1 strain through peripheral routes.…”

Section: Introductionmentioning

confidence: 99%

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Potential for transmission of sporadic Creutzfeldt–Jakob disease through peripheral routes

Kobayashi

Munesue

Shimazaki

et al. 2021

Laboratory Investigation

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Five sporadic Creutzfeldt-Jakob disease (CJD) strains have been identified to date, based on differences in clinicopathological features of the patients, the biochemical properties of abnormal prion proteins, and transmission properties. Recent advances in our knowledge about iatrogenic transmission of sporadic CJD have raised the possibility that the infectivity of sporadic CJD strains through peripheral routes is different from that of intracranial infection.To test this possibility, here we assessed systematically the infectivity of sporadic CJD strains through the peripheral route for the first time using a mouse model expressing human prion protein. Although the infectivity of the V2 and M1 sporadic CJD strains is almost the same in intracerebral transmission studies, the V2 strain infected more efficiently than the M1 strain through the peripheral route. The other sporadic CJD strains examined lacked infectivity. Of note, both the V2 and M1 strains showed preference for mice with the valine homozygosity at the PRNP polymorphic codon. These results indicate that the V2 strain is the most infectious sporadic CJD strain for infection through peripheral routes. In addition, these findings raise the possibility that individuals with the valine homozygosity at the PRNP polymorphic codon might have higher risks of infection through peripheral routes compared with the methionine homozygotes. Thus, preventive measures against the transmission of the V2 sporadic CJD strain will be important for the eradication of iatrogenic CJD transmission through peripheral routes.

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Section: Resultssupporting

confidence: 82%

Section: Transmission Experimentsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Potential for transmission of sporadic Creutzfeldt–Jakob disease through peripheral routes

Kobayashi

Munesue

Shimazaki

et al. 2021

Laboratory Investigation

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“…PrP Sc was purified from human brains or mouse brains as described. 19 Briefly, brain tissues were homogenized in 2 mL of lysis buffer (100 mmol/L Tris-HCl pH 8.0, 10 mmol/L NaCl, 10 mmol/L MgCl 2 , 2% Triton X-100, and 25 U/mL DNase I; all from Takara Bio, Shiga, Japan) and digested with collagenase (1 mg/200 mg tissue; FUJIFILM Wako Pure Chemical Corporation, Osaka, Japan) overnight at room temperature. Collagenase digestion disrupts the connective tissue and improves the accessibility of detergents and/or proteinase K to PrP Sc .…”

Section: Prp Sc Purificationmentioning

confidence: 99%

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

Kobayashi

Iwasaki

Takao

et al. 2019

The American Journal of Pathology

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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 proteinehumanized 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.

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“…PrP Sc was purified from the mouse brain as described. 23 Briefly, brain tissues were homogenized in 2 mL of lysis buffer [100 mmol/L tris-HCl, pH 8.0, 10 mmol/L NaCl, 10 mmol/L MgCl 2 , 2% Triton X-100, and 25 U/mL DNase I (Takara Bio, Shiga, Japan)] and digested with collagenase (1 mg/200 mg tissue) (FUJIFILM Wako Pure Chemical Corp.) overnight at room temperature. Collagenase digestion disrupts the connective tissue and improves the accessibility of detergents and/or proteinase K (PK) to PrP Sc .…”

Section: Prp Sc Purificationmentioning

confidence: 99%

Ganglioside Synthase Knockout Reduces Prion Disease Incubation Time in Mouse Models

Kobayashi

Shimazaki

et al. 2019

The American Journal of Pathology

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Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases

Cited by 5 publications

References 32 publications

Potential for transmission of sporadic Creutzfeldt–Jakob disease through peripheral routes

Potential for transmission of sporadic Creutzfeldt–Jakob disease through peripheral routes

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

Ganglioside Synthase Knockout Reduces Prion Disease Incubation Time in Mouse Models

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