Acta Neuropathologica

, Volume 96, Issue 3, pp 279–286

Mitochondrial dysfunction induced by oxidative stress in the brains of hamsters infected with the 263 K scrapie agent

  • Seung-Il Choi
  • W.-K. Ju
  • Eun-Kyoung Choi
  • Jin Kim
  • Ho-Zoo Lea
  • R. I. Carp
  • H. M. Wisniewski
  • Y.-S. Kim
Regular paper

DOI: 10.1007/s004010050895

Cite this article as:
Choi, SI., Ju, WK., Choi, EK. et al. Acta Neuropathol (1998) 96: 279. doi:10.1007/s004010050895

Abstract

Scrapie, one of the prion diseases, is a transmissible neurodegenerative disease of sheep and other animals. Clinical symptoms of prion diseases are characterized by a long latent period, followed by progressive ataxia, tremor, and death. To study the induction of neurodegeneration during scrapie infection, we have analyzed the activities of various antioxidant enzymes and mitochondrial enzymes in cerebral cortex, brain stem, and cerebellum of scrapie-infected hamsters. The activity of mitochondrial Mn-superoxide dismutase (SOD) was decreased, while the activities of cytosolic Cu/Zn-SOD and catalase were not altered in infected brains. The activities of glutathione peroxidase and glutathione reductase were increased in scrapie-infected hamsters. The decreased activity of Mn-SOD might result in increasing oxidative stress in the mitochondria of infected brain; this concept is supported by our findings of a high level of lipid peroxidation, and low levels of ATPase and cytochrome c oxidase activity in the infected cerebral mitochondria. In addition, structural abnormalities of mitochondria have been observed in the neurons of hippocampus and cerebral cortex of infected brain. These results suggest that mitochondrial dysfunction caused by oxidative stress gives rise to neurodegeneration in prion disease.

Key words ScrapieOxidative stressMitochondrial dysfunctionNeurodegeneration

Copyright information

© Springer-Verlag Berlin Heidelberg 1998

Authors and Affiliations

  • Seung-Il Choi
    • 1
  • W.-K. Ju
    • 1
  • Eun-Kyoung Choi
    • 1
  • Jin Kim
    • 2
  • Ho-Zoo Lea
    • 4
  • R. I. Carp
    • 3
  • H. M. Wisniewski
    • 3
  • Y.-S. Kim
    • 1
  1. 1.Institute of Environment & Life Science and Department of Microbiology, College of Medicine, Hallym University, Chunchon 200–702, Korea e-mail: de1951@sun.hallym.ac.kr, Tel.: 82-361-240-1951, Fax: 82-361-241-3422KR
  2. 2.Department of Anatomy, Catholic University, Medical College, Seoul, KoreaKR
  3. 3.New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USAIS
  4. 4.Division of Biological Sciences, Kangweon National University, Chunchon, KoreaKR