Cell death and autophagy in prion diseases (transmissible spongiform encephalopathies)

David R. Brown; Beata Sikorska; Byron Caughey; Paul Brown

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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1/2008
vol. 46

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abstract:

Cell death and autophagy in prion diseases (transmissible spongiform encephalopathies)

David R. Brown

,

Beata Sikorska

,

Byron Caughey

,

Paul Brown

Folia Neuropathol 2008; 46 (1): 1-25

Online publish date: 2008/03/21

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Neuronal autophagy, like apoptosis, is one of the mechanisms of programmed cell death. In this review, 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 transmissible spongiform encephalopathies (TSEs). 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.

keywords:

Cell death and autophagy in prion diseases (transmissible spongiform encephalopathies)

David R. Brown , Beata Sikorska , Byron Caughey , Paul Brown

autophagy, apoptosis, prion diseases, neurons, ultrastructure

David R. Brown

,

Beata Sikorska

,

Byron Caughey

,

Paul Brown