Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
- 24 October 1996
- journal article
- research article
- Published by Springer Nature in Nature
- Vol. 383 (6602) , 685-690
- https://doi.org/10.1038/383685a0
Abstract
Strains of transmissible spongiform encephalopathies are distinguished by differing physico-chemical 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.Keywords
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