The diverse roles of mononuclear phagocytes in prion disease pathogenesis

Abstract

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are neurological diseases that can be transmitted through a number of different routes. A wide range of mammalian species are affected by the disease. After peripheral exposure, some TSE agents accumulate in lymphoid tissues at an early stage of disease prior to spreading to the nerves and the brain. Much research has focused on identifying the cells and molecules involved in the transmission of TSE agents from the site of exposure to the brain and several crucial cell types have been associated with this process. The identification of the key cells that influence the different stages of disease transmission might identify targets for therapeutic intervention. This review highlights the involvement of mononuclear phagocytes in TSE disease. Current data suggest these cells may exhibit a diverse range of roles in TSE disease from the transport or destruction of TSE agents in lymphoid tissues, to mediators or protectors of neuropathology in the brain.

Keywords: :

• dendritic cells
• macrophages
• mononuclear phagocytes
• prion transmission
• PrP^Sc
• TSE

Disclosure of Potential Conflicts of Interest

The authors declare no financial conflict of interest.

Acknowledgments

The authors wish to thank Bob Fleming and the Pathology Services Group (The Roslin Institute and R(D)SVS, University of Edinburgh, UK) for excellent technical support. This work was supported by project and Institute Strategic Programme Grant funding from the Biotechnology and Biological Sciences Research Council.