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Cell biology of prions and prionoids: a status report


Aguzzi, Adriano; Lakkaraju, Asvin K K (2016). Cell biology of prions and prionoids: a status report. Trends in Cell Biology, 26(1):40-51.

Abstract

The coalescence of proteins into highly ordered aggregates is a hallmark of protein misfolding disorders (PMDs), which, when affecting the central nervous system, lead to progressive neurodegeneration. Although the chemical identity and the topology of each culprit protein are unique, the principles governing aggregation and propagation are strikingly stereotypical. It is now clear that such protein aggregates can spread from cell to cell and eventually affect entire organ systems - similarly to prion diseases. However, because most aggregates are not found to transmit between individuals, they are not infectious sensu strictiori. Therefore, they are not identical to prions and we prefer to define them as 'prionoids'. Here we review recent advances in understanding the toxicity of protein aggregation affecting the brain.

The coalescence of proteins into highly ordered aggregates is a hallmark of protein misfolding disorders (PMDs), which, when affecting the central nervous system, lead to progressive neurodegeneration. Although the chemical identity and the topology of each culprit protein are unique, the principles governing aggregation and propagation are strikingly stereotypical. It is now clear that such protein aggregates can spread from cell to cell and eventually affect entire organ systems - similarly to prion diseases. However, because most aggregates are not found to transmit between individuals, they are not infectious sensu strictiori. Therefore, they are not identical to prions and we prefer to define them as 'prionoids'. Here we review recent advances in understanding the toxicity of protein aggregation affecting the brain.

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9 citations in Web of Science®
11 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:2016
Deposited On:12 Nov 2015 09:30
Last Modified:05 Apr 2016 19:30
Publisher:Elsevier
ISSN:0962-8924
Publisher DOI:https://doi.org/10.1016/j.tcb.2015.08.007
PubMed ID:26455408
Permanent URL: https://doi.org/10.5167/uzh-114214

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