MOLECULAR ANALYSIS OF PRION STRAIN VARIATION AND THE ETIOLOGY OF NEW VARIANT CJD

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 str ain characteristics distinct from other types of CJD and which resembl e those of BSE transmitted to mice, domestic cat and macaque, consiste nt with BSE being the source of this new disease. Strain characteristi cs revealed here suggest that the prion protein may itself encode dise ase phenotype.