Prion Diseases pp 145-158 | Cite as

Cell Death and Autophagy in Prion Diseases

  • Pawel P. LiberskiEmail author
Part of the Neuromethods book series (NM, volume 129)


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.

Key words

Autophagy Apoptosis Prion diseases Neurons Ultrastructure 



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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© Springer Science+Business Media LLC 2017

Authors and Affiliations

  1. 1.Department of Molecular Pathology and NeuropathologyMedical University LodzLodzPoland

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