α-synuclein interacts with SOD1 and promotes its oligomerization

Mol Neurodegener. 2015 Dec 8:10:66. doi: 10.1186/s13024-015-0062-3.

Abstract

Background: Parkinson's disease (PD) and amyotrophic lateral sclerosis (ALS) are both neurodegenerative diseases leading to impaired execution of movement. α-Synuclein plays a central role in the pathogenesis of PD whereas Cu, Zn superoxide dismutase (SOD1) is a key player in a subset of familial ALS cases. Under pathological conditions both α-synuclein and SOD1 form oligomers and fibrils. In this study we investigated the possible molecular interaction of α-synuclein and SOD1 and its functional and pathological relevance.

Results: Using a protein-fragment complementation approach and co-IP, we found that α-synuclein and SOD1 physically interact in living cells, human erythrocytes and mouse brain tissue. Additionally, our data show that disease related mutations in α-synuclein (A30P, A53T) and SOD1 (G85R, G93A) modify the binding of α-synuclein to SOD1. Notably, α-synuclein accelerates SOD1 oligomerization independent of SOD1 activity.

Conclusion: This study provides evidence for a novel interaction of α-synuclein and SOD1 that might be relevant for neurodegenerative diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / metabolism*
  • Animals
  • Brain / metabolism*
  • Humans
  • Mice, Transgenic
  • Mutation / genetics
  • Parkinson Disease / metabolism
  • Protein Multimerization
  • Superoxide Dismutase / metabolism*
  • Superoxide Dismutase-1
  • alpha-Synuclein / metabolism*

Substances

  • SNCA protein, human
  • SOD1 protein, human
  • Snca protein, mouse
  • alpha-Synuclein
  • Sod1 protein, mouse
  • Superoxide Dismutase
  • Superoxide Dismutase-1

Supplementary concepts

  • Amyotrophic lateral sclerosis 1