Insights into prion strains and neurotoxicity.
Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are caused by prions and affect humans and many animal species. It is now widely accepted that the infectious agent that causes TSEs is PrP(Sc), an aggregated moiety of the host-derived membrane glycolipoprotein PrP(C). Although PrP(C) is encoded by the host genome, prions themselves encipher many phenotypic TSE variants, known as prion strains. Prion strains are TSE isolates that, after inoculation into distinct hosts, cause disease with consistent characteristics, such as incubation period, distinct patterns of PrP(Sc) distribution and spongiosis and relative severity of the spongiform changes in the brain. The existence of such strains poses a fascinating challenge to prion research.
Aguzzi
Adriano
A
Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland. adriano.aguzzi@usz.ch
Heikenwalder
Mathias
M
Polymenidou
Magdalini
M
D000595:Amino Acid Sequence; D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D017124:Conserved Sequence; D007562:Creutzfeldt-Jakob Syndrome / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D018562:Disease Transmission, Infectious; D005544:Forecasting; D014644:Genetic Variation; D006031:Glycosylation; D006801:Humans; D015994:Incidence; D008954:Models, Biological; D008969:Molecular Sequence Data; D018620:PrPSc Proteins / Q000737:chemistry / Q000235:genetics / Q000302:isolation & purification / Q000378:metabolism / Q000472:pathogenicity* / Q000633:toxicity*; D017096:Prion Diseases / Q000145:classification / Q000453:epidemiology / Q000209:etiology / Q000473:pathology / Q000635:transmission / Q000662:veterinary; D011328:Prions / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism / Q000472:pathogenicity* / Q000633:toxicity*; D021382:Protein Sorting Signals; D017433:Protein Structure, Secondary; D012608:Scrapie / Q000473:pathology / Q000635:transmission; D013045:Species Specificity; D014018:Tissue Distribution
D018620:PrPSc Proteins; D011328:Prions; D021382:Protein Sorting Signals