Alpha synuclein aggregation drives ferroptosis: an interplay of iron, calcium and lipid peroxidation

Cell Death Differ. 2020 Oct;27(10):2781-2796. doi: 10.1038/s41418-020-0542-z. Epub 2020 Apr 27.

Abstract

Protein aggregation and abnormal lipid homeostasis are both implicated in neurodegeneration through unknown mechanisms. Here we demonstrate that aggregate-membrane interaction is critical to induce a form of cell death called ferroptosis. Importantly, the aggregate-membrane interaction that drives ferroptosis depends both on the conformational structure of the aggregate, as well as the oxidation state of the lipid membrane. We generated human stem cell-derived models of synucleinopathy, characterized by the intracellular formation of α-synuclein aggregates that bind to membranes. In human iPSC-derived neurons with SNCA triplication, physiological concentrations of glutamate and dopamine induce abnormal calcium signaling owing to the incorporation of excess α-synuclein oligomers into membranes, leading to altered membrane conductance and abnormal calcium influx. α-synuclein oligomers further induce lipid peroxidation. Targeted inhibition of lipid peroxidation prevents the aggregate-membrane interaction, abolishes aberrant calcium fluxes, and restores physiological calcium signaling. Inhibition of lipid peroxidation, and reduction of iron-dependent accumulation of free radicals, further prevents oligomer-induced toxicity in human neurons. In summary, we report that peroxidation of polyunsaturated fatty acids underlies the incorporation of β-sheet-rich aggregates into the membranes, and that additionally induces neuronal death. This suggests a role for ferroptosis in Parkinson's disease, and highlights a new mechanism by which lipid peroxidation causes cell death.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Calcium / metabolism*
  • Cells, Cultured
  • Ferroptosis*
  • Human Embryonic Stem Cells
  • Humans
  • Induced Pluripotent Stem Cells
  • Iron / metabolism*
  • Lipid Peroxidation*
  • Parkinson Disease* / metabolism
  • Parkinson Disease* / pathology
  • alpha-Synuclein / metabolism*

Substances

  • alpha-Synuclein
  • Iron
  • Calcium