Abnormalities of the electron transport chain in idiopathic parkinson's disease

TLDR

Idiopathic Parkinson’s disease shows a low‑level familial association but is not Mendelian, and components of the electron transport chain are inherited non‑Mendelian. The study evaluated electron‑transport‑chain catalytic activities in platelet mitochondria from idiopathic Parkinson’s patients to test whether a complex I defect, potentially mitochondrial‑genome derived, contributes to disease pathogenesis. All ten patients exhibited markedly reduced complex I activity, with a modest decrease in succinate‑cytochrome c oxidoreductase activity.

Abstract

Abstract Idiopathic Parkinson's disease may have a low‐level familial association but does not follow mendelian patterns of inheritance. Since inheritance of some components of the electron transport chain is nonmendelian and since inhibition of the electron transport chain with the toxin 1‐methy1‐4‐phenyl‐1,2,3,6‐tetrahydropyridine models Parkinson's disease in humans and animals, we evaluated catalytic activities of the electron transport chain in platelet mitochondria purified from patients with idiopathic Parkinson's disease. All 10 patients studied had significant reductions of complex I (NADH:ubiquinone oxidoreductase) activity. Succinate:cytochrome c oxidoreductase activity was less strikingly reduced. We hypothesize that the complex I abnormality may have an etiological role in the pathogenesis of Parkinson's disease and that this defect may be derived via the mitochondrial genome.