Structural characterization of toxic oligomers that are kinetically trapped during α-synuclein fibril formation

Proc Natl Acad Sci U S A. 2015 Apr 21;112(16):E1994-2003. doi: 10.1073/pnas.1421204112. Epub 2015 Apr 8.

Abstract

We describe the isolation and detailed structural characterization of stable toxic oligomers of α-synuclein that have accumulated during the process of amyloid formation. Our approach has allowed us to identify distinct subgroups of oligomers and to probe their molecular architectures by using cryo-electron microscopy (cryoEM) image reconstruction techniques. Although the oligomers exist in a range of sizes, with different extents and nature of β-sheet content and exposed hydrophobicity, they all possess a hollow cylindrical architecture with similarities to certain types of amyloid fibril, suggesting that the accumulation of at least some forms of amyloid oligomers is likely to be a consequence of very slow rates of rearrangement of their β-sheet structures. Our findings reveal the inherent multiplicity of the process of protein misfolding and the key role the β-sheet geometry acquired in the early stages of the self-assembly process plays in dictating the kinetic stability and the pathological nature of individual oligomeric species.

Keywords: amyloid aggregation; cryoelectron microscopy; neurodegeneration; protein misfolding; toxic oligomer.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloid / chemistry*
  • Cryoelectron Microscopy
  • Hydrophobic and Hydrophilic Interactions
  • Imaging, Three-Dimensional
  • Models, Molecular
  • Molecular Weight
  • Protein Multimerization*
  • Protein Structure, Secondary
  • alpha-Synuclein / chemistry*
  • alpha-Synuclein / toxicity*
  • alpha-Synuclein / ultrastructure

Substances

  • Amyloid
  • alpha-Synuclein