Seeking a mechanism for the toxicity of oligomeric α-synuclein

Biomolecules. 2015 Mar 25;5(2):282-305. doi: 10.3390/biom5020282.

Abstract

In a number of neurological diseases including Parkinson's disease (PD), α-synuclein is aberrantly folded, forming abnormal oligomers, and amyloid fibrils within nerve cells. Strong evidence exists for the toxicity of increased production and aggregation of α-synuclein in vivo. The toxicity of α-synuclein is popularly attributed to the formation of "toxic oligomers": a heterogenous and poorly characterized group of conformers that may share common molecular features. This review presents the available evidence on the properties of α-synuclein oligomers and the potential molecular mechanisms of their cellular disruption. Toxic α-synuclein oligomers may impact cells in a number of ways, including the disruption of membranes, mitochondrial depolarization, cytoskeleton changes, impairment of protein clearance pathways, and enhanced oxidative stress. We also examine the relationship between α-synuclein toxic oligomers and amyloid fibrils, in the light of recent studies that paint a more complex picture of α-synuclein toxicity. Finally, methods of studying and manipulating oligomers within cells are described.

Publication types

  • Review

MeSH terms

  • Amyloid / metabolism
  • Animals
  • Humans
  • Mutation
  • Neurodegenerative Diseases / metabolism*
  • Protein Aggregation, Pathological / metabolism*
  • Protein Multimerization
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism*

Substances

  • Amyloid
  • alpha-Synuclein