Abstract
We report here on the evolution of view on the nature of transmissible spongiform encephalopathies or prion disease. While the nosological position of these diseases is well understood, the nature of the agent is still a matter of dispute. There is no doubt, however, that the gene for PrP plays a major role in the whole group of neurodegenerations.
Publication types
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Historical Article
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Amino Acid Substitution
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Amyloid / genetics
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Brain / pathology
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Child
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Codon / genetics
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Creutzfeldt-Jakob Syndrome / genetics
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Creutzfeldt-Jakob Syndrome / history
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Creutzfeldt-Jakob Syndrome / pathology
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Female
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Gerstmann-Straussler-Scheinker Disease / genetics
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Gerstmann-Straussler-Scheinker Disease / pathology
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History, 19th Century
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Humans
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Kuru / etiology
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Kuru / pathology
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Kuru / transmission
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Male
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Middle Aged
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Point Mutation
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Prion Diseases / etiology
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Prion Diseases / pathology*
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Prion Diseases / transmission
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Prion Proteins
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Prions / pathogenicity
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Protein Precursors / genetics
Substances
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Amyloid
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Codon
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PRNP protein, human
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Prion Proteins
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Prions
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Protein Precursors