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

PLoS Pathog. 2021 Jun 28;17(6):e1009703. doi: 10.1371/journal.ppat.1009703. eCollection 2021 Jun.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cricetinae
  • Dynamic Light Scattering / methods*
  • Hydrodynamics
  • Mice
  • Photometry / methods*
  • PrPSc Proteins / analysis*
  • PrPSc Proteins / chemistry*
  • Protein Structure, Quaternary

Substances

  • PrPSc Proteins

Grants and funding

This research was supported in part by Genome Canada in support of the Systems Biology and Molecular Ecology of Chronic Wasting Disease project (DM), the Alberta Prion Research Institute in collaboration with the Alberta Livestock and Meat Agency (Exploration IV, 201600016/2016A003R) (VS), the CJD Foundation (LC, VS), and the Alberta Synergies in Alzheimer’s and Related Disorders (SynAD) program which is funded by the Alzheimer Society of Alberta and Northwest Territories through their Hope for Tomorrow program and the University Hospital Foundation (VS, LC). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.