Cells and prions: A license to replicate

Edited by Per Hammarström

Received 23 March 2009; received in revised form 1 June 2009; accepted 9 June 2009. published online 15 June 2009.

Abstract

Prion diseases are neurodegenerative, infectious disorders characterized by the aggregation of a misfolded isoform of the cellular prion protein (PrPC). The infectious agent – termed prion – is mainly composed of misfolded PrPSc. In addition to the central nervous system prions can colonize secondary lymphoid organs and inflammatory foci. Follicular dendritic cells are important extraneural sites of prion replication. However, recent data point to a broader range of cell types that can replicate prions. Here, we review the state of the art in regards to peripheral prion replication, neuroinvasion and the determinants of prion replication competence.

Keywords: Prion replication competence, Follicular dendritic cell, Stromal inflammatory cell, Neuroinvasion, Mesenchymal progenitor cell

a Department of Pathology, Institutes of Neuropathology, University Hospital Zurich, CH 8091, Switzerland

b Amylod Center, Fondazione IRCCS Policlinico San Matteo, Department of Internal Medicine, University of Pavia, Pavia, Italy

Corresponding author. Fax: +41 (44) 255 4402.

PII: S0014-5793(09)00460-8

doi:10.1016/j.febslet.2009.06.014

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