Fulltext

Immune system and peripheral nerves in propagation of prions to CNS

Aguzzi, Adriano ; Heppner, Frank L. ; Heikenwalder, Mathias ; Prinz, Marco ; Mertz, Kirsten ; Seeger, Harald ; Glatzel, Markus

In: British Medical Bulletin, 2003, vol. 66, no. 1, p. 141-159

Add to personal list
    Title
    • Immune system and peripheral nerves in propagation of prions to CNS
    Author
    • Aguzzi, Adriano. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    • Heppner, Frank L.. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    • Heikenwalder, Mathias. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    • Prinz, Marco. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    • Mertz, Kirsten. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    • Seeger, Harald. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    • Glatzel, Markus. Institute of Neuropathology, Universitätsspital Zürich, Switzerland
    Document Type
    • Postprint
    Language
    • English
    Published in
    • British Medical Bulletin, 2003, vol. 66, no. 1, p. 141-159. Oxford University Press
    Other Version
    Classification
    • Health
    OAI-PMH Identifier
    • oai:doc.rero.ch:296040
    Summary
    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