NR AVFE
AU Aguzzi,A.; Heikenwalder,M.
TI Pathogenesis of prion diseases: current status and future outlook.
QU Nature Reviews. Microbiology 2006 Oct; 4(10): 765-75
PT journal article; review
AB The prion, a conformational variant of a host protein, is the infectious particle responsible for transmissible spongiform encephalopathy (TSE), a fatal neurodegenerative disease of humans and animals. The principal target of prion pathology is the brain, yet most TSEs also display prion replication at extra-cerebral locations, including secondary lymphoid organs and sites of chronic inflammation. Despite significant progress in our understanding of this infectious agent, many fundamental questions relating to the nature of the prion, including the mechanism of replication and the molecular events underlying brain damage, remain unanswered. Here we focus on the unresolved issues pertaining to prion pathogenesis, particularly on the role played by the immune system.
ZR 159
MH Animals; Humans; PrPsc Proteins/chemistry/*metabolism; Prion Diseases/*physiopathology/transmission/veterinary; Research Support, Non-U.S. Gov't; Ruminants/metabolism
AD Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland. adriano.aguzzi@usz.ch
SP englisch
PO England