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Prions and peripheral nerves: a deadly rendezvous


Heikenwalder, M; Julius, C; Aguzzi, A (2007). Prions and peripheral nerves: a deadly rendezvous. Journal of Neuroscience Research, 85(12):2714-2725.

Abstract

The infectious particle causing transmissible spongiform encephalopathy (TSE), a fatal neurodegenerative disease of humans and animals, has been termed prion. Its major component is an aggregated variant of the cellular prion protein, PrP(C). The main target of prion pathology is the central nervous system (CNS), yet most prion diseases are initiated or accompanied by prion replication at extracerebral locations, including secondary lymphoid organs, muscle and, in some instances, blood. How do prions travel from the periphery into the CNS? Is this an active or a passive process and does neuronal prion transport explain the long incubation times in prion diseases? Alternatively, if prion infectivity arises spontaneously in the CNS, as believed from sporadic Creutzfeldt-Jakob patients, how do prions manage to travel from the CNS into the periphery (e.g., spleen, muscle) of the infected host? The mechanisms of neuronal prion transport from the periphery into the CNS or vice versa are heavily investigated and debated but poorly understood. Although research in the past has accumulated knowledge on prion progression from the periphery to the brain, we are far from understanding the molecular mechanisms of neuronal prion transport.

The infectious particle causing transmissible spongiform encephalopathy (TSE), a fatal neurodegenerative disease of humans and animals, has been termed prion. Its major component is an aggregated variant of the cellular prion protein, PrP(C). The main target of prion pathology is the central nervous system (CNS), yet most prion diseases are initiated or accompanied by prion replication at extracerebral locations, including secondary lymphoid organs, muscle and, in some instances, blood. How do prions travel from the periphery into the CNS? Is this an active or a passive process and does neuronal prion transport explain the long incubation times in prion diseases? Alternatively, if prion infectivity arises spontaneously in the CNS, as believed from sporadic Creutzfeldt-Jakob patients, how do prions manage to travel from the CNS into the periphery (e.g., spleen, muscle) of the infected host? The mechanisms of neuronal prion transport from the periphery into the CNS or vice versa are heavily investigated and debated but poorly understood. Although research in the past has accumulated knowledge on prion progression from the periphery to the brain, we are far from understanding the molecular mechanisms of neuronal prion transport.

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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:2007
Deposited On:02 Jul 2012 12:44
Last Modified:05 Apr 2016 15:43
Publisher:Wiley-Blackwell
ISSN:0360-4012
Publisher DOI:10.1002/jnr.21246
PubMed ID:17393538
Permanent URL: http://doi.org/10.5167/uzh-60828

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