- Prof. KITAMOTO, Tetsuyuki
- Assist.Prof. TAKEUCNI, Atsuko
- KITAMOTO, Tetsuyuki
Objective- The Division of CJD Science and Technology aims at developing methods to prevent prion diseases. There are two directions of our main research.
① Establishment of animal models for human prion diseases
Bioassays for human prion have been performed conventionally using wild-type mice and required long incubation period (more than 600 days). We established knock-in mice expressing human prion protein and shortened the incubation period to less than 150 days.
Furthermore, we developed a new assay for human prion using the follicular dendritic cells of knock-in mice and shortened the observation period to 14 days. The transmission study using humanized knock-in mice revealed novel properties of prion, e.g., traceback and heterozygous inhibition.
② Elucidation of the conversion mechanism of prion protein
We are studying which domains of prion protein are essential for the conversion.
Selected Awards, articles and books
1)Human PrPSc 219K is converted to PrPSc but shows heterozygous inhibition in vCJD infection J. Biol. Chem. In Press 2008 2)Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain J. Biol. Chem. 282(41): 30022-30028 2007
3)vCJD prion acquires altered virulence through trans-species infection Biochem. Biophys. Res. Commun. 342(1): 293-299 2006
4)Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions Biochem. Biophys. Res. Commun. 294(2): 280-286 2002
5)Thr but Asn of the N-glycosylation sites of PrP is indispensable for its misfolding Biochem. Biophys. Res. Commun. 369(4): 1195-1198 2008
Prion, Creutzfeldt-Jakob disease, Bovine spongiform encephalopathy, Knock-in mouse, Transmission, Amino acid substitution, Polymorphism, PMCA, Neuropathology, Brain
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