Abstract
The pathological prion protein, PrPSc, displays various sizes of aggregates. In this study, we investigated the conformation, aggregation stability and proteinase K (PK)-sensitivity of small and large PrPSc aggregates of mouse-adapted prion strains. We showed that small PrPSc aggregates, previously thought to be PK-sensitive, are resistant to PK digestion. Furthermore, we showed that small PrPSc aggregates of the Chandler scrapie strain have greater resistance to PK digestion and aggregation-denaturation than large PrPSc aggregates of this strain. We conclude that this strain consists of heterogeneous PrPSc.
Highlights
Prion diseases, such as scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and Creutzfeldt-Jakob disease (CJD) in humans, are transmissible neurodegenerative disorders [1].They are characterized by the accumulation of pathogenic isoforms of prion protein (PrPSc), which is a major component of the infectious agent—the prion [1]
We discovered that small PrPSc aggregates of the Chandler scrapie strain have characteristics distinct from those of other prion strains
Using Western blotting, strong PrP signals were observed from fractions 1 and 2 from uninfected mice, and a faint signal was observed from fractions 3 and 4
Summary
Prion diseases, such as scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and Creutzfeldt-Jakob disease (CJD) in humans, are transmissible neurodegenerative disorders [1] They are characterized by the accumulation of pathogenic isoforms of prion protein (PrPSc), which is a major component of the infectious agent—the prion [1]. Some strains differ in their PrPSc properties, e.g., the electrophoretic mobilities associated with different cleavage sites of protease digestion [6], relative glycoform ratios [7] and immunoreactivities against conformationspecific antibodies [8,9]. These findings indicate that prion strain characteristics might be encoded in the structure and/or conformation of PrPSc
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