Immunology of Prion Protein and Prions

Neil A. Mabbott*

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter (peer-reviewed)peer-review

Abstract / Description of output

Many natural prion diseases are acquired peripherally, such as following the oral consumption of contaminated food or pasture. After peripheral exposure many prion isolates initially accumulate to high levels within the host's secondary lymphoid tissues. The replication of prions within these tissues is essential for their efficient spread to the brain where they ultimately cause neurodegeneration. This chapter describes our current understanding of the critical tissues, cells, and molecules which the prions exploit to mediate their efficient propagation from the site of exposure (such as the intestine) to the brain. Interactions between the immune system and prions are not only restricted to the secondary lymphoid tissues. Therefore, an account of how the activation status of the microglial in the brain can also influence progression of prion disease pathogenesis is provided. Prion disease susceptibility may also be influenced by additional factors such as chronic inflammation, coinfection with other pathogens, and aging. Finally, the potential for immunotherapy to provide a means of safe and effective prophylactic or therapeutic intervention in these currently untreatable diseases is considered.

Original languageEnglish
Title of host publicationPrion Protein
EditorsGuiseppe Legname, Silvia Vanni
Publication statusE-pub ahead of print - 31 Jul 2017

Publication series

NameProgress in Molecular Biology and Translational Science

Keywords / Materials (for Non-textual outputs)

  • B cells
  • Conventional dendritic cells
  • Follicular dendritic cells
  • Gut-associated lymphoid tissues
  • Lymph nodes
  • M cells
  • Macrophages
  • Microglia
  • Neuroinvasion
  • Peyer's patches
  • Prion protein
  • Prions
  • Spleen
  • Transmissible spongiform encephalopathies


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