Efficient prion transmission via the intranasal and aerosolic route

Completed Research Project

Title / Titel: Efficient prion transmission via the intranasal and aerosolic route

Summary / Zusammenfassung: Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative
diseases of humans and animals. The underlying infectious agent, the prion, was
shown to accumulate not only in the central nervous system (CNS) but also in
secondary lymphoid organs of affected hosts. Prions can colonize hosts by a variety
of extracerebral routes, including parenteral injection, transdermal administration
after skin scarification, and oral administration. Up to date prions were not considered
to be transmissible by aerial routes. Here we have investigated the transmissibility
potential of prions administered intranasally or by aerosols. Various transgenic mouse
models expressing the cellular prion protein (PrPC) in specific compartments or cells
of the brain (e.g. exclusively in the CNS) were investigated to identify the cellular and
molecular mechanism(s) of prion invasion via the intranasal or aerosolic route.
Results of this study identify prion aerosols or prions administered intranasally as a
startlingly efficacious pathway of prion transmission, and call for appropriate
revisions of prion-related biosafety guidelines.

Keywords / Suchbegriffe: Prion disease, aerosols, intranasal prion infection

Project Leadership and Contacts / Projektleitung und Kontakte

Prof. Dr. Adriano Aguzzi (Project Leader)

Funding Source(s) / Unterstützt durch: Nachwuchsförderungskredit der Universität Zürich, Other Public Sources (e.g. Federal or Cantonal Agencies), Foundation