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. 2012;8(5):e1002651.
doi: 10.1371/journal.ppat.1002651. Epub 2012 May 3.

Five questions on prion diseases

Adriano Aguzzi  1 , Caihong Zhu
Affiliations

Five questions on prion diseases

Adriano Aguzzi et al. PLoS Pathog. 2012.
No abstract available

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. The cascade of prion entry, peripheral replication, neuroinvasion, and neurodegeneration.
After peripheral exposure, prions colonize and replicate in secondary lymphoid organs (SLOs) like spleen, Payer's patches, lymph nodes, and tonsils. FDCs are the main sites accumulating prions in SLOs. B cell-derived LTs and TNF facilitate prion accumulation by supporting development and maintenance of FDCs. Dedifferentiation of FDCs by LTβR-Ig delays neuroinvasion, whereas repetitive immunization accelerates prion pathogenesis. Prions reach the central nervous system (CNS) through autonomic nerves, directly after intracerebral inoculation, or via aerosols through immune-independent pathways. In the brain, prions replicate but are also cleared by microglia after opsonisation by astrocyte-borne Mfge8. Prion deposition comes about when PrPSc production exceeds PrPSc clearance.
See this image and copyright information in PMC

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