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Pathogenesis of prion diseases: current status and future outlook
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Journal Article
Pathogenesis of prion diseases: current status and future outlook
2006
Overview
Key Points
Prion diseases are progressive, transmissible neurodegenerative disorders with an invariably fatal outcome. Prions, the infectious agent of prion diseases, accumulate in the central nervous system, in organs of the secondary lymphoid system and in blood.
Examples of prion diseases include bovine spongiform encephalopathy (BSE) in cows, scrapie in sheep and goat, chronic wasting disease in deer and elk, and sporadic and variant Creutzfeldt–Jakob disease in humans.
Neuronal cytotoxicity of PrP
Sc
depends on the expression of PrP
C
. Evidence indicates that the conversion of PrP
C
to PrP
Sc
is deleterious, but the mechanisms of neural degeneration are still unclear.
In this article, we describe the role of the immune system in prion diseases and review our current understanding of cellular and molecular mechanisms involved in peripheral prion replication and transport.
Several prion diseases are transmitted by peripheral prion uptake (for example, ingestion of prion-contaminated food). After prion uptake, a replication phase occurs in lymphoid tissue before neuroinvasion.
In the peripheral regions of the host, the abnormally folded, aggregated PrP
Sc
, is amplified by cells of the immune system (for example, follicular dendritic cells) in the germinal centres, located in B-cell follicles of the spleen or lymph nodes.
Depletion of mature follicular dendritic cells delays the development of prion disease following intraperitoneal inoculation. This could form the basis of a post-exposure prophylactic strategy.
Recent findings indicate that chronic inflammation can induce the deposition of prion infectivity in organs previously believed to be prion free.
In recent years, significant progress has been made in our understanding of the biology of prions, yet many fundamental questions remain unanswered. Aguzzi and Heikenwalder discuss some of these unanswered questions, focusing on the role of the immune system in prion pathogenesis.
The prion, a conformational variant of a host protein, is the infectious particle responsible for transmissible spongiform encephalopathy (TSE), a fatal neurodegenerative disease of humans and animals. The principal target of prion pathology is the brain, yet most TSEs also display prion replication at extra-cerebral locations, including secondary lymphoid organs and sites of chronic inflammation. Despite significant progress in our understanding of this infectious agent, many fundamental questions relating to the nature of the prion, including the mechanism of replication and the molecular events underlying brain damage, remain unanswered. Here we focus on the unresolved issues pertaining to prion pathogenesis, particularly on the role played by the immune system.
Publisher
Nature Publishing Group UK,Nature Publishing Group
Subject
