An in vitro model of Parkinson's disease: linking mitochondrial impairment to altered alpha-synuclein metabolism and oxidative damage

J Neurosci. 2002 Aug 15;22(16):7006-15. doi: 10.1523/JNEUROSCI.22-16-07006.2002.

Abstract

Chronic systemic complex I inhibition caused by rotenone exposure induces features of Parkinson's disease (PD) in rats, including selective nigrostriatal dopaminergic degeneration and formation of ubiquitin- and alpha-synuclein-positive inclusions (Betarbet et al., 2000). To determine underlying mechanisms of rotenone-induced cell death, we developed a chronic in vitro model based on treating human neuroblastoma cells with 5 nm rotenone for 1-4 weeks. For up to 4 weeks, cells grown in the presence of rotenone had normal morphology and growth kinetics, but at this time point, approximately 5% of cells began to undergo apoptosis. Short-term rotenone treatment (1 week) elevated soluble alpha-synuclein protein levels without changing message levels, suggesting that alpha-synuclein degradation was retarded. Chronic rotenone exposure (4 weeks) increased levels of SDS-insoluble alpha-synuclein and ubiquitin. After a latency of >2 weeks, rotenone-treated cells showed evidence of oxidative stress, including loss of glutathione and increased oxidative DNA and protein damage. Chronic rotenone treatment (4 weeks) caused a slight elevation in basal apoptosis and markedly sensitized cells to further oxidative challenge. In response to H2O2, there was cytochrome c release from mitochondria, caspase-3 activation, and apoptosis, all of which occurred earlier and to a much greater extent in rotenone-treated cells; caspase inhibition provided substantial protection. These studies indicate that chronic low-grade complex I inhibition caused by rotenone exposure induces accumulation and aggregation of alpha-synuclein and ubiquitin, progressive oxidative damage, and caspase-dependent death, mechanisms that may be central to PD pathogenesis.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Antiparkinson Agents / pharmacology
  • Apoptosis / drug effects
  • Caspase 3
  • Caspase Inhibitors
  • Caspases / metabolism
  • Cell Respiration / drug effects
  • Cytochrome c Group / metabolism
  • DNA Damage / drug effects
  • Drug Synergism
  • Electron Transport Complex I
  • Enzyme Inhibitors / pharmacology
  • Glutathione / metabolism
  • Humans
  • Hydrogen Peroxide / pharmacology
  • Mitochondria / drug effects*
  • Mitochondria / metabolism
  • NADH, NADPH Oxidoreductases / antagonists & inhibitors*
  • Nerve Tissue Proteins / metabolism
  • Neuroblastoma / drug therapy
  • Neuroblastoma / metabolism
  • Neurons / drug effects*
  • Neurons / metabolism
  • Neurons / pathology
  • Oxidants / pharmacology
  • Oxidation-Reduction / drug effects
  • Oxidative Stress / drug effects
  • Parkinson Disease / metabolism*
  • Parkinson Disease / pathology
  • Parkinson Disease, Secondary / chemically induced
  • Rotenone / pharmacology*
  • Synucleins
  • Time
  • Tumor Cells, Cultured
  • Ubiquitin / metabolism
  • Uncoupling Agents / pharmacology
  • alpha-Synuclein

Substances

  • Antiparkinson Agents
  • Caspase Inhibitors
  • Cytochrome c Group
  • Enzyme Inhibitors
  • Nerve Tissue Proteins
  • Oxidants
  • SNCA protein, human
  • Synucleins
  • Ubiquitin
  • Uncoupling Agents
  • alpha-Synuclein
  • Rotenone
  • Hydrogen Peroxide
  • NADH, NADPH Oxidoreductases
  • CASP3 protein, human
  • Caspase 3
  • Caspases
  • Electron Transport Complex I
  • Glutathione