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Cell Death and Autophagy in Prion Diseases

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Prion Diseases

Part of the book series: Neuromethods ((NM,volume 129))

Abstract

Neuronal autophagy, like apoptosis, is one of the mechanisms of programmed cell death. In this chapter, we summarize current information about autophagy in naturally occurring and experimentally induced scrapie, Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome, against the broad background of neural degenerations in prion diseases. Typically a sequence of events is observed: from a part of the neuronal cytoplasm sequestrated by concentric arrays of double membranes (phagophores) through the enclosure of the cytoplasm and membrane proliferation to a final transformation of the large area of the cytoplasm into a collection of autophagic vacuoles of different sizes. These autophagic vacuoles form not only in neuronal perikarya but also in neurites and synapses. On the basis of ultrastructural studies, we suggest that autophagy may play a major role in transmissible spongiform encephalopathies and may even participate in the formation of spongiform change.

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Acknowledgments

This chapter is supported in part by Healthy Aging Research Center (HARC, FP7-REGPOT-2012-2013-1). Ms. Ewa Skarzynska, Mr. Ryszard Kurczewski, Ms. Elzbieta Naganska, Ms. Leokadia Romanska, and Mr. Kazimierz Smoktunowicz are kindly acknowledged for skilful technical assistance.

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  1. Department of Molecular Pathology and Neuropathology, Medical University Lodz, Czechoslowacka street 8/10, Lodz, PL 92-216, Poland

    Pawel P. Liberski

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Correspondence to Pawel P. Liberski .

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  1. Department of Molecular Pathology, Medical University of Lodz, Lodz, Poland

    Pawel P. Liberski

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Liberski, P.P. (2017). Cell Death and Autophagy in Prion Diseases. In: Liberski, P. (eds) Prion Diseases. Neuromethods, vol 129. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7211-1_8

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  • DOI: https://doi.org/10.1007/978-1-4939-7211-1_8

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