Defined α-synuclein prion-like molecular assemblies spreading in cell culture

BMC Neurosci. 2014 Jun 4:15:69. doi: 10.1186/1471-2202-15-69.

Abstract

Background: α-Synuclein (α-syn) plays a central role in the pathogenesis of synucleinopathies, a group of neurodegenerative disorders that includes Parkinson disease, dementia with Lewy bodies and multiple system atrophy. Several findings from cell culture and mouse experiments suggest intercellular α-syn transfer.

Results: Through a methodology used to obtain synthetic mammalian prions, we tested whether recombinant human α-syn amyloids can promote prion-like accumulation in neuronal cell lines in vitro. A single exposure to amyloid fibrils of human α-syn was sufficient to induce aggregation of endogenous α-syn in human neuroblastoma SH-SY5Y cells. Remarkably, endogenous wild-type α-syn was sufficient for the formation of these aggregates, and overexpression of the protein was not required.

Conclusions: Our results provide compelling evidence that endogenous α-syn can accumulate in cell culture after a single exposure to exogenous α-syn short amyloid fibrils. Importantly, using α-syn short amyloid fibrils as seed, endogenous α-syn aggregates and accumulates over several passages in cell culture, providing an excellent tool for potential therapeutic screening of pathogenic α-syn aggregates.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line
  • Humans
  • Macromolecular Substances / metabolism*
  • Mice
  • Neurons / metabolism*
  • Prions / classification*
  • Prions / metabolism*
  • alpha-Synuclein / classification*
  • alpha-Synuclein / metabolism*

Substances

  • Macromolecular Substances
  • Prions
  • alpha-Synuclein