| First Author | Taguchi Y | Year | 2003 |
| Journal | Am J Pathol | Volume | 163 |
| Issue | 6 | Pages | 2585-93 |
| PubMed ID | 14633630 | Mgi Jnum | J:86603 |
| Mgi Id | MGI:2680851 | Doi | 10.1016/S0002-9440(10)63613-9 |
| Citation | Taguchi Y, et al. (2003) Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol 163(6):2585-93 |
| abstractText | Mice to which human prions efficiently transmit in short incubation periods are valuable not only as research tools of human prions but also as reliable diagnostic tools. We recently produced a line of knock-in mouse expressing a unique human-mouse chimeric PrP (Ki-ChM mouse), which has mouse-specific residues practically only at the C-terminal part after posttranslational modification, and here we attempted transmission of various human prions to assess the susceptibility profile of the mouse. Susceptibility varied considerably depending on prions inoculated: highly susceptible to MM1 and MV1 types of sporadic Creutzfeldt-Jakob disease (CJD), developing disease within approximately 150 days, familial CJD with M232R mutation, and dura graft-associated CJD (dCJD) without amyloid plaque; less susceptible to MM2-type sporadic CJD and variant CJD, with some mice lacking any sign of transmission; and totally resistant to VV2 type sporadic CJD and dCJD with amyloid plaque. The rather short incubation time achieved by Ki-ChM mice suggests new approaches to produce mice that develop prion disease with very short incubation periods. We compared the characteristic susceptibility profile of Ki-ChM with those of other precedent transgenic mice and discussed, including the prospects in developing genetically engineered mice susceptible to human prions. |
