Header

UZH-Logo

Maintenance Infos

Immune system and peripheral nerves in propagation of prions to CNS.


Aguzzi, A; Heppner, F L; Heikenwalder, M; Prinz, M; Mertz, K; Seeger, H; Glatzel, M (2003). Immune system and peripheral nerves in propagation of prions to CNS. British Medical Bulletin, 66(1):141-159.

Abstract

Prions are not only unique in the way they replicate. Also the sequence of events triggered by peripheral prion infection, generically termed 'peripheral pathogenesis', sets prions aside from all other known pathogens. Whereas most bacteria, parasites, and viruses trigger innate and adaptive immune responses, the mammalian immune system appears to be remarkably oblivious to prions. Transmissible spongiform encephalopathies (TSEs) do not go along with inflammatory infiltrates, and antibodies to the prion protein are not typically raised during the course of the disease. On the other hand, there is conspicuous involvement of lymphoid organs, which accumulate sizeable concentrations of the infectious agent early during disease. Moreover, various states of immune deficiency can abolish peripheral pathogenesis and prevent 'take' of infection when prions are administered to peripheral sites. Here, we critically re-visit the current evidence for an involvement of the immune system in prion diseases, and will attempt to trace the elaborate mechanisms by which prions, upon entry into the body from peripheral sites, reach the brain.

Abstract

Prions are not only unique in the way they replicate. Also the sequence of events triggered by peripheral prion infection, generically termed 'peripheral pathogenesis', sets prions aside from all other known pathogens. Whereas most bacteria, parasites, and viruses trigger innate and adaptive immune responses, the mammalian immune system appears to be remarkably oblivious to prions. Transmissible spongiform encephalopathies (TSEs) do not go along with inflammatory infiltrates, and antibodies to the prion protein are not typically raised during the course of the disease. On the other hand, there is conspicuous involvement of lymphoid organs, which accumulate sizeable concentrations of the infectious agent early during disease. Moreover, various states of immune deficiency can abolish peripheral pathogenesis and prevent 'take' of infection when prions are administered to peripheral sites. Here, we critically re-visit the current evidence for an involvement of the immune system in prion diseases, and will attempt to trace the elaborate mechanisms by which prions, upon entry into the body from peripheral sites, reach the brain.

Statistics

Citations

Dimensions.ai Metrics
47 citations in Web of Science®
49 citations in Scopus®
90 citations in Microsoft Academic
Google Scholar™

Altmetrics

Downloads

5 downloads since deposited on 11 Feb 2008
5 downloads since 12 months
Detailed statistics

Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Uncontrolled Keywords:General Medicine
Language:English
Date:2003
Deposited On:11 Feb 2008 12:26
Last Modified:17 Aug 2018 10:09
Publisher:Oxford University Press
ISSN:0007-1420
OA Status:Green
Publisher DOI:https://doi.org/10.1093/bmb/66.1.141
PubMed ID:14522856

Download

Download PDF  'Immune system and peripheral nerves in propagation of prions to CNS.'.
Preview
Content: Published Version
Language: English
Filetype: PDF (Nationallizenz 142-005)
Size: 214kB
View at publisher