title: Molecular biology of prions.
abstract: 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.
authors.affiliation: MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1N 3BG, UK. charles.weissmann@prion.ucl.ac.uk.
authors.forename: Charles
authors.initials: C
authors.lastname: Weissmann
authors.forename: Masato
authors.initials: M
authors.lastname: Enari
authors.forename: Peter-Christian
authors.initials: PC
authors.lastname: Klöhn
authors.forename: Daniela
authors.initials: D
authors.lastname: Rossi
authors.forename: Eckhard
authors.initials: E
authors.lastname: Flechsig
mesh_terms: D000818:Animals; D006801:Humans; D007049:Iatrogenic Disease; D017096:Prion Diseases / Q000235:genetics* / Q000635:transmission; D011328:Prions / Q000235:genetics* / Q000502:physiology*
chemical_list: D011328:Prions