Abstract
Strains of transmissible spongiform encephalopathies are distinguished by differing physicochemical properties of PrPSc, the disease-related isoform of prion protein, which can be maintained on transmission to transgenic mice. 'New variant' Creutzfeldt-Jakob disease (CJD) has strain characteristics distinct from other types of CJD and which resemble those of BSE transmitted to mice, domestic cat and macaque, consistent with BSE being the source of this new disease. Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Cats
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Creutzfeldt-Jakob Syndrome / classification
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Creutzfeldt-Jakob Syndrome / etiology*
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Creutzfeldt-Jakob Syndrome / transmission
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Encephalopathy, Bovine Spongiform / etiology
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Endopeptidase K / metabolism
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Genetic Variation
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Glycosylation
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Growth Hormone / administration & dosage
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Humans
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Macaca
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Mice
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Mice, Inbred C57BL
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Mice, Transgenic
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PrPC Proteins / chemistry
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PrPC Proteins / genetics
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PrPC Proteins / metabolism
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PrPSc Proteins / chemistry
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PrPSc Proteins / genetics
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PrPSc Proteins / metabolism
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Prions / chemistry
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Prions / classification
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Prions / genetics*
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Prions / metabolism
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Protein Conformation
Substances
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PrPC Proteins
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PrPSc Proteins
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Prions
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Growth Hormone
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Endopeptidase K