NR AEPS
AU Glatzel,M.; Aguzzi,A.
TI The shifting biology of prions
QU Brain Research. Brain Research Reviews 2001 Oct; 36(2-3): 241-8
PT journal article; review; review, tutorial
AB Transmissible spongiform encephalopathies (TSEs), or prion diseases, are rare fatal neurodegenerative diseases of humans and animals. Although some TSEs, like scrapie in sheep, have been known to exist for centuries, bovine spongiform encephalopathy (BSE) was recognized only 15 years ago. New variant Creutzfeldt-Jakob disease (nvCJD) of humans is probably caused by consumption of BSE-infected materials. The nature of the infectious agent is not fully elucidated, but substantial evidence suggests that it is devoid of nucleic acids and consists at least in part of an abnormal form of a host protein termed PrPc. Despite their rarity, prion diseases have become an important topic in public health and basic research because of the connection between nvCJD and BSE and also because of the unusual biological attributes of the infectious agent.
ZR 42
MH Animal; Central Nervous System/*immunology/pathology/physiopathology; Creutzfeldt-Jakob Syndrome/genetics/pathology/physiopathology; Diagnostic Tests, Routine; Human; Mice; Mice, Transgenic; Prion Diseases/*genetics/pathology/physiopathology; Prions/*genetics/metabolism/pathogenicity; Scrapie/genetics/pathology/physiopathology; Support, Non-U.S. Gov't
AD Department of Pathology, Institute of Neuropathology, Schmelzbergstrasse 12, University Hospital Zürich, CH-8091, Zürich, Switzerland.
SP englisch
PO Niederlande