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Neuronal autophagy in experimental Creutzfeldt-Jakob's disease

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Summary

We report an experimental model of Creutzfeldt-Jakob's disease (CJD) in mice leading to the formation of giant autophagic vacuoles (AV) in neurons of the cerebral cortex. These AV appear at the end of the incubation period (4–6 months post-inoculation), together with spongy changes and clinical symptoms. Autophagy, a process of intracellular digestion of cell constituents by the lysosomal compartment, is known in many cell types, where it plays a role both in the physiological turnover and in pathological processes and is involved in protein metabolism. The same also occurs in neurons. Here autophagy is known to occur in the normal state and leads to residual bodies called lipofuscin granules. An increase in lipofuscin is known to occur in human and experimental CJD. Therefore, an increase in autophagy and in AV can be expected. In our experimental model, the activation of neuronal autophagy may be related to an alteration in neuronal protein metabolism.

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Authors and Affiliations

  1. Institut für Hirnforschung der Universität, Calwerstrasse 3, D-7400, Tübingen, Federal Republic of Germany

    J. W. Boellaard

  2. Neurologisches Institut der Universtität, Deutschordenstrasse 46, D-6000, Frankfurt am Main 71, Federal Republic of Germany

    W. Schlote

  3. Department of Neuropathology, Neurological Institute, Faculty of Medicine, Kyushu University, 812, Fukuoka, Japan

    J. Tateishi

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  1. J. W. Boellaard
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  2. W. Schlote
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  3. J. Tateishi
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Boellaard, J.W., Schlote, W. & Tateishi, J. Neuronal autophagy in experimental Creutzfeldt-Jakob's disease. Acta Neuropathol 78, 410–418 (1989). https://doi.org/10.1007/BF00688178

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  • DOI: https://doi.org/10.1007/BF00688178

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