FEBS Letters
Volume 583, Issue 16 , Pages 2674-2684, 20 August 2009

Cells and prions: A license to replicate

Edited by Per Hammarström

  • Mario Nuvolone

      Affiliations

    • Department of Pathology, Institutes of Neuropathology, University Hospital Zurich, CH 8091, Switzerland
    • Amylod Center, Fondazione IRCCS Policlinico San Matteo, Department of Internal Medicine, University of Pavia, Pavia, Italy
    • ,
  • Adriano Aguzzi

      Affiliations

    • Department of Pathology, Institutes of Neuropathology, University Hospital Zurich, CH 8091, Switzerland
    • ,
  • Mathias Heikenwalder

      Affiliations

    • Department of Pathology, Institutes of Neuropathology, University Hospital Zurich, CH 8091, Switzerland
    • Corresponding Author InformationCorresponding author. Fax: +41 (44) 255 4402.

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

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PII: S0014-5793(09)00460-8

doi:10.1016/j.febslet.2009.06.014

FEBS Letters
Volume 583, Issue 16 , Pages 2674-2684, 20 August 2009

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