Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel

  • Authors:
    • Chi-Kyeong Kim
    • Akikazu Sakudo
    • Yojiro Taniuchi
    • Kazuto Shigematsu
    • Chung-Boo Kang
    • Keiichi Saeki
    • Yoshitsugu Matsumoto
    • Suehiro Sakaguchi
    • Shigeyoshi Itohara
    • Takashi Onodera
  • View Affiliations

  • Published online on: August 1, 2007     https://doi.org/10.3892/ijmm.20.2.169
  • Pages: 169-176
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Abstract

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.

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August 2007
Volume 20 Issue 2

Print ISSN: 1107-3756
Online ISSN:1791-244X

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Spandidos Publications style
Kim C, Sakudo A, Taniuchi Y, Shigematsu K, Kang C, Saeki K, Matsumoto Y, Sakaguchi S, Itohara S, Onodera T, Onodera T, et al: Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel. Int J Mol Med 20: 169-176, 2007.
APA
Kim, C., Sakudo, A., Taniuchi, Y., Shigematsu, K., Kang, C., Saeki, K. ... Onodera, T. (2007). Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel. International Journal of Molecular Medicine, 20, 169-176. https://doi.org/10.3892/ijmm.20.2.169
MLA
Kim, C., Sakudo, A., Taniuchi, Y., Shigematsu, K., Kang, C., Saeki, K., Matsumoto, Y., Sakaguchi, S., Itohara, S., Onodera, T."Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel". International Journal of Molecular Medicine 20.2 (2007): 169-176.
Chicago
Kim, C., Sakudo, A., Taniuchi, Y., Shigematsu, K., Kang, C., Saeki, K., Matsumoto, Y., Sakaguchi, S., Itohara, S., Onodera, T."Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel". International Journal of Molecular Medicine 20, no. 2 (2007): 169-176. https://doi.org/10.3892/ijmm.20.2.169