Prions and the lymphoreticular system

Philos Trans R Soc Lond B Biol Sci. 2001 Feb 28;356(1406):177-84. doi: 10.1098/rstb.2000.0763.

Abstract

Following intracerebral or peripheral inoculation of mice with scrapie prions, infectivity accumulates first in the spleen and only later in the brain. In the spleen of scrapie-infected mice, prions were found in association with T and B lymphocytes and to a somewhat lesser degree with the stroma, which contains the follicular dendritic cells (FDCs) but not with non-B, non-T cells; strikingly, no infectivity was found in lymphocytes from blood of the same mice. Transgenic PrP knockout mice expressing PrP restricted to either B or T lymphocytes show no prion replication in the lymphoreticular system. Therefore, splenic lymphocytes either acquire prions from another source or replicate them in dependency on other PrP-expressing cells. The essential role of FDCs in prion replication in spleen was shown by treating mice with soluble lymphotoxin-beta receptor, which led to disappearance of mature FDCs from the spleen and concomitantly abolished splenic prion accumulation and retarded neuroinvasion following intraperitoneal scrapie inoculation.

Publication types

  • Review

MeSH terms

  • Animals
  • Dendritic Cells
  • Humans
  • Lymphatic System / physiology*
  • Mice
  • Mice, Knockout
  • Mononuclear Phagocyte System / physiology*
  • Peptide Fragments / genetics
  • Prions / genetics
  • Prions / metabolism*
  • Spleen / metabolism

Substances

  • Peptide Fragments
  • Prions
  • prion protein (105-132)