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Asymmetric-flow field-flow fractionation of prions reveals a strain-specific continuum of quaternary structures with protease resistance developing at a hydrodynamic radius of 15 nm.
Cortez, Leonardo M; Nemani, Satish K; Duque Velásquez, Camilo; Sriraman, Aishwarya; Wang, YongLiang; Wille, Holger; McKenzie, Debbie; Sim, Valerie L.
Afiliación
  • Cortez LM; Centre for Prions and Protein Folding Diseases, Edmonton, Alberta, Canada.
  • Nemani SK; Department of Medicine, Division of Neurology, University of Alberta, Edmonton, Alberta, Canada.
  • Duque Velásquez C; Centre for Prions and Protein Folding Diseases, Edmonton, Alberta, Canada.
  • Sriraman A; Department of Medicine, Division of Neurology, University of Alberta, Edmonton, Alberta, Canada.
  • Wang Y; Centre for Prions and Protein Folding Diseases, Edmonton, Alberta, Canada.
  • Wille H; Department of Biological Sciences, University of Alberta, Edmonton, Alberta, Canada.
  • McKenzie D; Centre for Prions and Protein Folding Diseases, Edmonton, Alberta, Canada.
  • Sim VL; Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada.
PLoS Pathog ; 17(6): e1009703, 2021 06.
Article en En | MEDLINE | ID: mdl-34181702
Prion diseases are transmissible neurodegenerative disorders that affect mammals, including humans. The central molecular event is the conversion of cellular prion glycoprotein, PrPC, into a plethora of assemblies, PrPSc, associated with disease. Distinct phenotypes of disease led to the concept of prion strains, which are associated with distinct PrPSc structures. However, the degree to which intra- and inter-strain PrPSc heterogeneity contributes to disease pathogenesis remains unclear. Addressing this question requires the precise isolation and characterization of all PrPSc subpopulations from the prion-infected brains. Until now, this has been challenging. We used asymmetric-flow field-flow fractionation (AF4) to isolate all PrPSc subpopulations from brains of hamsters infected with three prion strains: Hyper (HY) and 263K, which produce almost identical phenotypes, and Drowsy (DY), a strain with a distinct presentation. In-line dynamic and multi-angle light scattering (DLS/MALS) data provided accurate measurements of particle sizes and estimation of the shape and number of PrPSc particles. We found that each strain had a continuum of PrPSc assemblies, with strong correlation between PrPSc quaternary structure and phenotype. HY and 263K were enriched with large, protease-resistant PrPSc aggregates, whereas DY consisted primarily of smaller, more protease-sensitive aggregates. For all strains, a transition from protease-sensitive to protease-resistant PrPSc took place at a hydrodynamic radius (Rh) of 15 nm and was accompanied by a change in glycosylation and seeding activity. Our results show that the combination of AF4 with in-line MALS/DLS is a powerful tool for analyzing PrPSc subpopulations and demonstrate that while PrPSc quaternary structure is a major contributor to PrPSc structural heterogeneity, a fundamental change, likely in secondary/tertiary structure, prevents PrPSc particles from maintaining proteinase K resistance below an Rh of 15 nm, regardless of strain. This results in two biochemically distinctive subpopulations, the proportion, seeding activity, and stability of which correlate with prion strain phenotype.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fotometría / Proteínas PrPSc / Dispersión Dinámica de Luz Límite: Animals Idioma: En Revista: PLoS Pathog Año: 2021 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fotometría / Proteínas PrPSc / Dispersión Dinámica de Luz Límite: Animals Idioma: En Revista: PLoS Pathog Año: 2021 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Estados Unidos