NR APGF
AU Chesebro,B.
TI Introduction to the transmissible spongiform encephalopathies or prion diseases
QU British Medical Bulletin 2003; 66: 1-20
PT journal article; review; review, tutorial
AB Sheep scrapie has been known for at least 200 years and was described as a transmissible disease over 100 years ago. Since then, three groups of transmissible spongiform encephalopathies or TSE diseases have been identified in humans including familial, infectious and sporadic types. The discovery of the prion protein (PrP) in the 1980s greatly accelerated knowledge of the biology and pathogenesis of TSE diseases as this protein was found to play a critical role in disease susceptibility and the TSE species-barrier and may also be a component of the infectious agent itself. Nevertheless, the nature of the TSE agents remains an enigma. Proof of the protein-only hypothesis may require generation of biologically active transmissible agent in a cell-free environment where a virus cannot replicate. Conversely, proof of a viral aetiology will require identification and isolation of a candidate virus. Further understanding of the structure of the disease-associated protease-resistant PrP should help elucidate the mechanism of PrP conversion from the normal to the abnormal form. Such information should open up new approaches to both diagnosis and therapy.
ZR 100
MH Animals; Brain/*metabolism; Cattle; Creutzfeldt-Jakob Syndrome/transmission; Deer; Encephalopathy, Bovine Spongiform/transmission; Human; Mice; Mice, Transgenic; Mink; Models, Animal; PrPsc Proteins/*metabolism; Prion Diseases/*etiology/transmission/virology; Prions/genetics; Scrapie/transmission; Sheep; Wasting Disease, Chronic/etiology
AD Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institutes of Allergy and Infectious Diseases, Hamilton, Montana 59840, USA
SP englisch
PO England