Parkinson's disease genetic mutations increase cell susceptibility to stress: mutant alpha-synuclein enhances H2O2- and Sin-1-induced cell death

Neurobiol Aging. 2007 Nov;28(11):1709-17. doi: 10.1016/j.neurobiolaging.2006.07.017. Epub 2006 Sep 15.

Abstract

Parkinson's disease (PD) is a progressive neurodegenerative movement disorder characterized by selective loss of dopaminergic neurons and the presence of Lewy bodies. Alpha-synuclein is a major component of Lewy bodies in sporadic PD, and genetic alterations in alpha-synuclein cause autosomal-dominant hereditary PD. The pathogenesis of PD remains incompletely understood, but it appears to involve both genetic susceptibility and environmental factors. Here we investigated the effect of alpha-synuclein expression on cell susceptibility to proteasome inhibition, oxidative and nitrative stresses by using a PC 12-Tet-off regulatory system. We found that inducible expression of A30P or A53T mutant alpha-synuclein decreased the proteasome activity, increased intracellular ROS levels, and enhanced lactacystin- and H2O2-induced cell death. Furthermore, 3-nitrotyrosine levels increased in cells expressing alpha-synuclein, and further increased after Sin-1 (a NO donor) treatment compared with untreated or treated non-induced cells. Expression of alpha-synuclein (mutant more than wild type) significantly enhances Sin-1 toxicity. These results indicate that genetic mutations in alpha-synuclein may increase neuronal vulnerability to cellular stress in aging and PD pathogenesis.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Cell Death / drug effects
  • Cell Death / physiology
  • Dose-Response Relationship, Drug
  • Genetic Predisposition to Disease*
  • Hydrogen Peroxide / toxicity*
  • Molsidomine / analogs & derivatives*
  • Molsidomine / toxicity
  • Mutation*
  • PC12 Cells
  • Parkinson Disease / genetics*
  • Parkinson Disease / metabolism
  • Parkinson Disease / pathology*
  • Rats
  • Reactive Oxygen Species / metabolism
  • alpha-Synuclein / biosynthesis
  • alpha-Synuclein / genetics*
  • alpha-Synuclein / physiology

Substances

  • Reactive Oxygen Species
  • alpha-Synuclein
  • linsidomine
  • Hydrogen Peroxide
  • Molsidomine