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Prions and the immune system: a journey through gut, spleen, and nerves.

Authors
  • Aguzzi, Adriano
Type
Published Article
Journal
Cell Biology of the B Cell Receptor
Publisher
Elsevier BV
Publication Date
Jan 01, 2003
Volume
81
Pages
123–171
Identifiers
PMID: 14711055
Source
Medline
License
Unknown

Abstract

For more than two decades it has been contended that prion infection does not elicit immune responses: transmissible spongiform encephalopathies do not go along with conspicuous inflammatory infiltrates, and antibodies to the prion protein are typically undetectable. Why is it, then, that prions accumulate in lymphoid organs, and that various states of immune deficiency prevent peripheral prion infection? This review revisits the current evidence of the involvement of the immune system in prion diseases, while attempting to trace the elaborate mechanisms by which peripherally administered prions invade the brain and ultimately cause damage. The investigation of these questions leads to unexpected detours, including the neurophysiology of lymphoid organs, and even the function of a prion protein homolog in male fertility.

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