Monitoring α-synuclein multimerization in vivo

FASEB J. 2019 Feb;33(2):2116-2131. doi: 10.1096/fj.201800148RRR. Epub 2018 Sep 25.

Abstract

The pathophysiology of Parkinson's disease is characterized by the abnormal accumulation of α-synuclein (α-Syn), eventually resulting in the formation of Lewy bodies and neurites in surviving neurons in the brain. Although α-Syn aggregation has been extensively studied in vitro, there is limited in vivo knowledge on α-Syn aggregation. Here, we used the powerful genetics of Drosophila melanogaster and developed an in vivo assay to monitor α-Syn accumulation by using a bimolecular fluorescence complementation assay. We found that both genetic and pharmacologic manipulations affected α-Syn accumulation. Interestingly, we also found that alterations in the cellular protein degradation mechanisms strongly influenced α-Syn accumulation. Administration of compounds identified as risk factors for Parkinson's disease, such as rotenone or heavy metal ions, had only mild or even no impact on α-Syn accumulation in vivo. Finally, we show that increasing phosphorylation of α-Syn at serine 129 enhances the accumulation and toxicity of α-Syn. Altogether, our study establishes a novel model to study α-Syn accumulation and illustrates the complexity of manipulating proteostasis in vivo.-Prasad, V., Wasser, Y., Hans, F., Goswami, A., Katona, I., Outeiro, T. F., Kahle, P. J., Schulz, J. B., Voigt, A. Monitoring α-synuclein multimerization in vivo.

Keywords: BiFC; Parkinson’s disease; protein aggregation; synucleinopathy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloid / chemistry*
  • Animals
  • Disease Models, Animal*
  • Drosophila melanogaster / growth & development
  • Drosophila melanogaster / metabolism*
  • Male
  • Phosphorylation
  • Protein Multimerization*
  • Reactive Oxygen Species / metabolism
  • Serine
  • alpha-Synuclein / chemistry*
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism*

Substances

  • Amyloid
  • Reactive Oxygen Species
  • alpha-Synuclein
  • Serine