Prion pathogenesis and secondary lymphoid organs (SLO): tracking the SLO spread of prions to the brain.

Prion pathogenesis and secondary lymphoid organs (SLO): tracking the SLO spread of prions to the brain.
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prion发病机理和继发性淋巴机构(SLO):追踪王室对大脑的SLO传播。

DOI:
10.4161/pri.20676
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发表时间:
2012-09
期刊:
影响因子:
2.3
通讯作者:
Mabbott NA
Mabbott NA
中科院分区:
生物学3区
文献类型:
--
作者:
Mabbott NA

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Prion diseases are subacute neurodegenerative diseases that affect humans and a range of domestic and free-ranging animal species. These diseases are characterized by the accumulation of PrPSc, an abnormally folded isoform of the cellular prion protein (PrPC), in affected tissues. The pathology during prion disease appears to occur almost exclusively within the central nervous system. The extensive neurodegeneration which occurs ultimately leads to the death of the host. An intriguing feature of the prion diseases, when compared with other protein-misfolding diseases, is their transmissibility. Following peripheral exposure, some prion diseases accumulate to high levels within lymphoid tissues. The replication of prions within lymphoid tissue has been shown to be important for the efficient spread of disease to the brain. This article describes recent progress in our understanding of the cellular mechanisms that influence the propagation of prions from peripheral sites of exposure (such as the lumen of the intestine) to the brain. A thorough understanding of these events will lead to the identification of important targets for therapeutic intervention, or alternatively, reveal additional processes that influence disease susceptibility to peripherally-acquired prion diseases.
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