Molecular biology of prions

• Authors: C. Weissmann , M. Enari , P.-C. Klohn , D. Rossi , E. Flechsig
• Languages of publication: EN

Abstracts

EN

The ?protein only? hypothesis holds that the infectious agent causing transmissible spongiform encephalopathies is a conformational isomer of PrP, a host protein predominantly expressed in brain and is strongly supported by many lines of evidence. Prion diseases are so far unique among conformational diseases in that they are transmissible, not only experimentally but also by natural routes, mainly by ingestion. The pathway of prions to the brain has been elucidated in outline. A striking feature of prions is their extraordinary resistance to conventional sterilisation procedures, and their capacity to bind to surfaces of metal and plastic without losing infectivity. This property, first observed in a clinical setting, is now being investigated in experimental settings, both in animals and in cell culture.

Keywords

EN

CONVERSION; INFECTION; PRION PROTEIN; PROPAGATION; STERILIZATION; STRAIN; TRANSMISSION

Discipline

• EXPERIMENTAL_BIOLOGY_&_MEDICINE: EXPERIMENTAL BIOLOGY & MEDICINE

• Publisher: Marceli Nencki Institute of Experimental Biology, Polish Academy of Sciences
• Journal: Acta Neurobiologiae Experimentalis
• Year: 2002
• Volume: 62
• Issue: 3
• Pages: 153-166

Contributors

author

C. Weissmann

author

M. Enari

author

P.-C. Klohn

author

D. Rossi

author

E. Flechsig

• Document Type: ARTICLE
• Publication order reference: Charles Weissmann, MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WCIN 3BG, UK