Lymph nodal prion replication and neuroinvasion in mice devoid of follicular dendritic cells

Proc Natl Acad Sci U S A. 2002 Jan 22;99(2):919-24. doi: 10.1073/pnas.022626399. Epub 2002 Jan 15.

Abstract

Variant Creutzfeldt-Jakob disease and scrapie are typically initiated by extracerebral exposure to prions, and exhibit early prion accumulation in germinal centers. Follicular dendritic cells (FDCs), whose development and maintenance in germinal centers depends on tumor necrosis factor (TNF) and lymphotoxin (LT) signaling, are thought to be indispensable for extraneural prion pathogenesis. Here, we administered prions intraperitoneally to mice deficient for TNF and LT signaling components. LT alpha(-/-), LT beta(-/-), LT betaR(-/-), and LT alpha(-/-) x TNFalpha(-/-) mice resisted infection and contained no infectivity in spleens and lymph nodes (when present). However, TNFR1(-/-), TNFR2(-/-), and some TNFalpha(-/-) mice developed scrapie similarly to wild-type mice. High prion titers were detected in lymph nodes, but not spleens, of TNFR1(-/-) and TNF alpha(-/-) mice despite absence of FDCs and germinal centers. Transfer of TNFR1(-/-) fetal liver cells into lethally irradiated Prnp(0/0) mice restored infectivity mainly in lymph nodes. Prion protein (PrP) colocalized with a minority of macrophages in tumor necrosis factor receptor (TNFR) 1(-/-) lymph nodes. Therefore, prion pathogenesis can be restricted to lymphoreticular subcompartments, and mature follicular dendritic cells are dispensable for this process. Macrophage subsets are plausible candidates for lymphoreticular prion pathogenesis and neuroinvasion in the absence of FDCs, and may represent a novel target for postexposure prophylaxis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antigens, CD / genetics
  • Chimera
  • Dendritic Cells, Follicular / pathology*
  • Humans
  • Lymph Nodes / metabolism*
  • Lymph Nodes / pathology*
  • Lymphotoxin beta Receptor
  • Lymphotoxin-alpha / genetics
  • Lymphotoxin-alpha / metabolism
  • Lymphotoxin-beta
  • Membrane Proteins / deficiency
  • Membrane Proteins / genetics
  • Mice
  • Mice, Knockout
  • Prion Diseases / etiology*
  • Prion Diseases / pathology
  • Prion Diseases / physiopathology
  • Prions / metabolism*
  • Prions / pathogenicity*
  • Receptors, Tumor Necrosis Factor / deficiency
  • Receptors, Tumor Necrosis Factor / genetics
  • Receptors, Tumor Necrosis Factor, Type I
  • Tumor Necrosis Factor-alpha / deficiency
  • Tumor Necrosis Factor-alpha / genetics

Substances

  • Antigens, CD
  • LTB protein, human
  • LTBR protein, human
  • Ltb protein, mouse
  • Ltbr protein, mouse
  • Lymphotoxin beta Receptor
  • Lymphotoxin-alpha
  • Lymphotoxin-beta
  • Membrane Proteins
  • Prions
  • Receptors, Tumor Necrosis Factor
  • Receptors, Tumor Necrosis Factor, Type I
  • Tumor Necrosis Factor-alpha