| First Author | Kim CK | Year | 2007 |
| Journal | Int J Mol Med | Volume | 20 |
| Issue | 2 | Pages | 169-76 |
| PubMed ID | 17611634 | Mgi Jnum | J:141915 |
| Mgi Id | MGI:3820021 | Citation | Kim CK, et al. (2007) Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel. Int J Mol Med 20(2):169-76 |
| abstractText | Several lines of prion protein gene (Prnp)-knockout mice such as ZrchI, ZrchII, Npu, Ngsk and Rcm0 have been generated. Of these, ZrchII, Ngsk and Rcm0 exhibit late-onset ataxia due to ectopic expression of Doppel (Dpl); a result of damage to the splicing acceptor of Prnp exon 3. Recently, we developed another line of Prnp-/- mice (Rikn), which was generated by gene targeting with more nucleotides by replacing intron 2 with the pgk-neo gene (cf. Ngsk Prnp-/- mice) and showed not only ataxia but also a lower olfactory sensitivity than the other Prnp-/- mouse line ZrchI at over 60 weeks of age. The histopathology of the elderly Rikn Prnp-/- mice showed mitral cell loss concomitantly observed with gliosis of astrocytes. Western blot analysis showed that Dpl was detected in the cerebrum, cerebellum and olfactory bulb of Rikn Prnp-/- mice, where aberrant histopathology was observed. Thus, mitral cell loss and gliosis induced by ectopic Dpl expression were probably associated with the late-onset olfactory deficits in Rikn Prnp-/- mice. |
