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Utilizing Time-Resolved Protein-Induced Fluorescence Enhancement to Identify Stable Local Conformations One α-Synuclein Monomer at a Time
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Direct Observation of Competing Prion Protein Fibril Populations with Distinct Structures and Kinetics.

Yuanzi Sun1, Kezia Jack1, Tiziana Ercolani1

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|February 21, 2023
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Summary

Prion protein (PrP) fibrils evolve through competing structural variants, akin to genetic quasispecies. This structural polymorphism allows prions to adapt and potentially evade therapies.

Keywords:
polymorphic amyloid structuresprion proteinprotein misfoldingreal-time kinetic measurementssuper-resolution microscopy

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Area of Science:

  • Neuroscience
  • Biochemistry
  • Molecular Biology

Background:

  • Prion diseases involve misfolded prion protein (PrP) forming self-propagating fibrils.
  • The evolutionary mechanisms of prion adaptation remain poorly understood.

Purpose of the Study:

  • To investigate the structural polymorphism and evolutionary dynamics of prion protein (PrP) fibrils.
  • To elucidate the mechanisms underlying prion adaptation and host-specific evolution.

Main Methods:

  • Utilized total internal reflection and transient amyloid binding super-resolution microscopy to monitor single PrP fibril structure and growth.
  • Analyzed distinct elongation kinetics and monomer incorporation mechanisms of competing fibril populations.

Main Results:

  • Identified at least two distinct PrP fibril populations emerging from homogeneous seeds.
  • Observed selective amplification and mutation of PrP conformers during fibril elongation.
  • Demonstrated differential monomer incorporation (unfolded vs. partially folded) and distinct elongation kinetics between fibril populations.

Conclusions:

  • Prion replication exhibits characteristics of molecular evolution, analogous to the quasispecies concept.
  • Polymorphic fibril populations compete and adapt, potentially influencing prion pathogenesis and therapeutic resistance.
  • Prions may represent quasispecies of structural isomorphs capable of evolving to new hosts.