Mitochondrial oxidative phosphorylation defects in parkinson's disease

Academic Article


  • Parkinson's disease has been associated with defects in oxidative phosphorylation (Oxphos). We analyzed mitochondria isolated from muscle biopsies of 6 patients with Parkinson's disease for deficiencies in Oxphos enzymes and for mutations in the mitochondrial DNA. Oxphos enzyme assays were compared to the 5 to 95% confidence intervals from 16 control subjects. Four patients had complex I defects, whereas 1 patient had a complex IV defect. A genetic basis for Parkinson's disease was suggested by the presence of affected relatives of 2 patients with Parkinson's disease. Known pathological mitochondrial DNA mutations (insertion‚Äźdeletions or point mutations) were not found. We conclude that Parkinson's disease is a systemic disorder of Oxphos, probably of a complex genetic etiology. Premature cell death in the nigrostriatal dopamine pathway could be due to energetic impairment and accentuated free radical generation caused by an Oxphos defect. Copyright ¬© 1991 American Neurological Association
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    Author List

  • Shoffner JM; Watts RL; Juncos JL; Torroni A; Wallace DC
  • Start Page

  • 332
  • End Page

  • 339
  • Volume

  • 30
  • Issue

  • 3