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Table 1 Abbreviations used throughout

From: The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease

Abbreviation Definition
PrPSc The abnormal, disease-associated form (generically termed the scrapie isoform, irrespective of the disease and species in which it occurs) which is characterised by increased β-pleated sheet content, decreased solubility and increased protease-resistance compared with PrPC as a result of refolding of the protein and self-aggregation.
PrPC Normal form of the prion protein as expressed in the central nervous system and other tissues.
PrPres Protease resistant core of PrPSc detected by western blotting following treatment with 50 μg/ml PK.
~8 kDa PrPres fragment Low molecular mass PrPres fragment of ~8 kDa detected by western blotting following treatment with 50 μg/ml PK in brain tissue from patients with VPSPr and some patients with GSS.
senPrPSc The component of PrPSc that is poorly resistant to protease treatment (i.e. >2.5 μg/ml PK)
CDI Conformation dependent immunoassay
GSS Gerstmann-Sträussler-Scheinker syndrome
GSS (~8 kDa PrPres) GSS case associated with a low molecular mass fragment of PrPres of approximately ~8 kDa when analysed by western blotting following proteinase K digestion.
GSS (type 1 PrPres) GSS case characterised by the presence of 20-30 kDa type 1 PrPres fragments in brain when analysed by western blotting following proteinase K digestion.