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Novel proteinaceous infectious particles cause scrapie.
TLDR
A new term "prion" is proposed to denote a small proteinaceous infectious particle which is resistant to inactivation by most procedures that modify nucleic acids.
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Nobel Lecture: Prions
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Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
TLDR
It is now feasible to determine whether mice devoid of PrPc can propagate prions and are susceptible to scrapie pathogenesis.
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Molecular biology of prion diseases
TLDR
Understanding prion diseases may advance investigations of other neurodegenerative disorders and of the processes by which neurons differentiate, function for decades, and then grow senescent.
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Eight prion strains have PrPSc molecules with different conformations
TLDR
It is indicated that each of the eight prion strains has a PrP sc molecule with a unique conformation and that the variation in incubation times is related to the relative protease sensitivity of PrPSc in each strain.
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Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel.
TLDR
Dpl is the first PrP-like protein to be described in mammals, and since Dpl seems to cause neurodegeneration similar to PrP, the linked expression of the Prnp and Prnd genes may play a previously unrecognized role in the pathogenesis of prion diseases or other illnesses.
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A transmembrane form of the prion protein in neurodegenerative disease.
TLDR
Aberrant regulation of protein biogenesis and topology at the endoplasmic reticulum can result in neurodegeneration.
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Identification of a protein that purifies with the scrapie prion.
TLDR
Initial results suggest that the amount of this protein correlates with the titer of the agent, and the resistance of thisprotein to digestion by proteinase K distinguished it from proteins of similar molecular weight found in normal hamster brain.
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Prion diseases and the BSE crisis.
TLDR
There is now considerable concern that bovine prions may have been passed to humans, resulting in a new form of CJD.
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Shattuck lecture--neurodegenerative diseases and prions.
  • S. Prusiner
  • Biology
    The New England journal of medicine
  • 17 May 2001
TLDR
Of all the laboratory research on neurodegenerative diseases, the studies that led to the discovery of prions have yielded the most unexpected findings.
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