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General Model of Prion Strains and Their Pathogenicity
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General Model of Prion Strains and Their Pathogenicity
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Journal Article
General Model of Prion Strains and Their Pathogenicity
2007
Overview
Prions are lethal mammalian pathogens composed of aggregated conformational isomers of a host-encoded glycoprotein and which appear to lack nucleic acids. Their unique biology, allied with the public-health risks posed by prion zoonoses such as bovine spongiform encephalopathy, has focused much attention on the molecular basis of prion propagation and the \"species barrier\" that controls cross-species transmission. Both are intimately linked to understanding how multiple prion \"strains\" are encoded by a protein-only agent. The underlying mechanisms are clearly of much wider importance, and analogous protein-based inheritance mechanisms are recognized in yeast and fungi. Recent advances suggest that prions themselves are not directly neurotoxic, but rather their propagation involves production of toxic species, which may be uncoupled from infectivity.
Publisher
American Association for the Advancement of Science,The American Association for the Advancement of Science
Subject
/ Biological and medical sciences
/ Bovine spongiform encephalopathy
/ Fundamental and applied biological sciences. Psychology
/ Humans
/ isomers
/ mammals
/ Mice
/ Non conventional transmissible agents
/ Prion Diseases - transmission
/ Prions
/ Prions - isolation & purification
/ PrPC Proteins - isolation & purification
/ PrPSc Proteins - isolation & purification
/ PrPSc Proteins - pathogenicity
/ Recombinant Proteins - chemistry
/ Review
/ risk
/ Yeasts
/ zoonoses
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