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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-deletion 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. |
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