Mitochondrial Disturbances in Parkinson’s Disease

  • Heinz Reichmann
  • Bernd Janetzky
  • Peter Riederer
Part of the Advances in Research on Neurodegeneration book series (ARN, volume 2)


In 1962, Luft defined mitochondrial myopathies to consist of typical morphological and biochemical abnormalities of the mitochondria in association with characteristic clinical symptoms (Luft et al., 1962). In 1981, Egger and co-workers introduced the term ‘mitochondrial cytopathy’ (Egger et al, 1981). In 1989, three groups independently reported on the possibility that Parkinson’s disease (PD) might belong to the mitochondrial cytopathies (Mizuno et al., 1989; Reichmann and Riederer, 1989; Schapira et al., 1989).


Mitochondrial Genome Substantia Nigra Myoclonic Epilepsy Mitochondrial Myopathy Parkinsonian Brain 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Ben-Shachar D, Eshel G, Riederer P, Youdim MBH (1992): Role of iron and iron chelation in dopaminergic-induced neurodegeneration: implication for Parkinson’s disease. Ann Neurol 32:S105–S110PubMedCrossRefGoogle Scholar
  2. Bindoff LA, Birch Machin M, Cartlidge NEF, Parker WDJ, Turnbull DM (1989): Mitochondrial function in Parkinson’s disease. Lancet 2:49PubMedCrossRefGoogle Scholar
  3. Dawson TM, Dawson VL, Snyder SH (1992): A novel messenger molecule in brain: the free radical, nitric oxide. Ann Neurol 32:297–311PubMedCrossRefGoogle Scholar
  4. Dexter DT, Wells FR, Agid F, Agid Y, Lees AJ, Jenner P, Marsden CD (1987): Increased nigral iron content in post mortem parkinsonian brain. Lancet ii: 1219–1220CrossRefGoogle Scholar
  5. Earle KM (1968): Studies on Parkinson’s disease including x-ray fluorescent spectroscopy of formalin fixed tissue. J Neuropathol Exp Neurol 27:1–14PubMedCrossRefGoogle Scholar
  6. Egger J, Lake BD, Wilson J (1981): Mitochondrial cytopathy. A multisystem disorder with ragged-red fibers on muscle biopsy. Arch Dis Child 56:741–752PubMedCrossRefGoogle Scholar
  7. Goto Y-I, Nonaka I, Horai S (1990): A mutation in the tRNALeu (UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature 348:651–653PubMedCrossRefGoogle Scholar
  8. Götz ME, Freyberger A, Riederer P (1990): Oxidative stress: a role in the pathogenesis of Parkinson’s disease. J Neural Transm (Suppl) 29:241–249Google Scholar
  9. Hess JF, Parisi MA, Bennett JL, Clayton DA (1991): Impairment of mitochondrial transcription termination by a point mutation associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature 351:236–239PubMedCrossRefGoogle Scholar
  10. Holt IJ, Harding AE, Morgan-Hughes JA (1988): Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies. Nature 331:717–719PubMedCrossRefGoogle Scholar
  11. Ikebe S, Tanaka M, Ohno K, Sato W, Hattori K, Kondo T, Mizuno Y, Ozawa T (1990): Increase of deleted mitochondrial DNA in the striatum in Parkinson’s disease and senescence. Biochem Biophys Res Commun 170:1044–1048PubMedCrossRefGoogle Scholar
  12. Jenner P, Dexter DT, Sian J, Schapira AHV, Marsden CD (1992): Oxidative stress as a cause of nigral cell death in Parkinson’s disease and incidental Lewy body disease. Ann Neurol 32:S82-S87PubMedCrossRefGoogle Scholar
  13. Krige D, Carroll MT, Cooper JM, Marsden CD, Schapira AHV (1992): Platelet mitochondrial function in Parkinson’s disease. Ann Neurol 32:782–788PubMedCrossRefGoogle Scholar
  14. Lestienne P, Nelson J, Riederer P, Jellinger K, Reichmann H (1990): Normal mitochondrial genome in brain from patients with Parkinson’s disease and complex I defect. J Neuroimmunol 55:1810–1812Google Scholar
  15. Lestienne P, Nelson J, Riederer P, Reichmann H, Jellinger K (1991): Mitochondrial DNA in postmortem brain from patients with Parkinson’s disease. J Neurochem 56:1819PubMedCrossRefGoogle Scholar
  16. Luft R, Ikkos D, Palmieri G, Ernster L, Afzelius B (1962): A case of severe hypermetabolism of nonthyroid origin with a defect in the maintenance of mitochondrial respiratory control. A correlated clinical, biochemical and morphological study. J Clin Invest 41:1776–1804PubMedCrossRefGoogle Scholar
  17. Mizuno Y, Sone N, Saitoh T (1987): Effects of 1-methyl-4-phenyl-1,2,3,6-tetrahy-dropyridine and 1-methyl-4-phenylpyridinium ion on activities of the enzymes in the electron transport system in mouse brain. J Neurochem 48:1787–1793PubMedCrossRefGoogle Scholar
  18. Mizuno Y, Suzuki K, Ohta S (1990): Postmortem changes in mitochondrial respiratory enzymes in brain and a preliminary observation in Parkinson’s disease. J Neurol Sci 96:49–57PubMedCrossRefGoogle Scholar
  19. Mizuno Y, Ohta S, Tanaka M, Takamiya S, Suzuki K, Sato T, Oya H, Ozawa T, Kagawa Y (1989): Deficiencies in complex I subunits of the respiratory chain in Parkinson’s disease. Biochem Biophys Res Commun 163:1450–1455PubMedCrossRefGoogle Scholar
  20. Moraes CT, DiMauro S, Zeviani M, Lombes A, Shanske S, Miranda A, Nakase H, Bonilla E, Werneck LC, Servidei S, Nonaka I, Koga Y, Spiro AJ, Brownell KW, Schmidt B, Schotland DL, Zupanc M, DeVivo DC, Schon E, Rowland LP (1989): Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome. N Engl J Med 320:1293–1299PubMedCrossRefGoogle Scholar
  21. Nicklas WJ, Vyas I, Heikkila RE (1985): Inhibition of NADH-linked oxidation in brain mitochondria by 1-methyl-4-phenylpyridine, a metabolite of the neurotoxic 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Life Sci 36:2503–2508PubMedCrossRefGoogle Scholar
  22. Pache T, Reichmann H (1990): On the stability of key enzymes of energy metabolism in muscle biopsies. Enzyme 43:183–187PubMedGoogle Scholar
  23. Parker WD, Boyson SJ, Parks JK (1989): Abnormalities of the electron transport chain in idiopathic Parkinson’s disease. Ann Neurol 26:719–723PubMedCrossRefGoogle Scholar
  24. Perry TL, Godin DV, Hansen S (1982): Parkinson’s disease: a disorder due to nigral glutathione deficiency. Neurosci Lett 33:305–310PubMedCrossRefGoogle Scholar
  25. Reichmann H, Riederer P (1989): Biochemische Analyse der Atmungskettenkomplexe verschiedener Hirnregionen von Patienten mit Morbus Parkinson. In: Symposium des BMFT Morbus Parkinson und andere Basalganglienerkrankun-gen, S. 44 (abstr.)Google Scholar
  26. Reichmann H, Riederer P, Seufert S (1990): Disturbances of the respiratory chain in brain from patients with Parkinson’s disease. Mov Disord 5:28Google Scholar
  27. Reichmann H, Hoppeler H, Mathieu-Costello O, von Bergen F, Pette D (1985): Biochemical and ultrastructural changes of skeletal muscle mitochondria after chronic electrical stimulation in rabbits. Eur J Physiol (Pflüger’s Arch) 404:1–9CrossRefGoogle Scholar
  28. Riederer P, Sofic E, Rausch WD, Schmidt B, Reynolds GP, Jellinger K, Youdim MBH (1989): Transition metals, ferritin, glutathione, and ascorbic acid in parkinsonian brains. J Neurochem 52:515–520PubMedCrossRefGoogle Scholar
  29. Rotig A, Colonna M, Bonnefont JP, Blanche S, Fischer A, Sandubray JM, Munnich A (1989): Mitochondrial DNA deletion in Pearson’s marrow/pancreas syndrome. Lancet 1:902–903PubMedCrossRefGoogle Scholar
  30. Schapira AVH, Cooper JM, Dexter D, Jenner P, Clark JB, Marsden CD (1989): Mitochondrial complex I deficiency in Parkinson’s disease (letter). Lancet i:1269Google Scholar
  31. Schapira AHV, Cooper JM, Dexter D, Clark JB, Jenner P, Marsden CD (1990): Mitochondrial complex I deficiency in Parkinson’s disease. J Neurochem 54:823–827PubMedCrossRefGoogle Scholar
  32. Shoffner JM, Watts RL, Juncos JL, Torroni A, Wallace DC (1991): Mitochondrial oxidative phosphorylation defects in Parkinson’s disease. Ann Neurol 30:332–339PubMedCrossRefGoogle Scholar
  33. Shoffner JM, Lott MT, Lezza AMS, Scibel P, Ballinger SW, Wallace DC (1990): Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNALys mutation. Cell 61:931–937PubMedCrossRefGoogle Scholar
  34. Sofie E, Riederer P, Heinsen H, Beckmann H, Reynolds GP, Hebenstreit G, Youdim MBH (1988): Increased iron (III) and total iron content in post mortem substantia nigra of parkinsonian brain. J Neural Transm 74:199–205CrossRefGoogle Scholar
  35. Wallace DC, Singh G, Lott MT, Hodge JA, Schurr TG, Lezza AMS, Elsas LJ, Nikoskelainen EK (1988): Mitochondrial DNA mutation associated with Leber’s hereditary optic neuropathy. Science 242:1427–1430PubMedCrossRefGoogle Scholar
  36. Wallace DC, Zheng X, Lott MT, Shoffner JM, Hodge JA, Kelley RI, Epstein CM, Hopkins LC (1990): Myoclonic epilepsy and ragged-red fiber disease (MERRF): genetic, pathophysiological and biochemical characterization of a mitochondrial DNA disease. Cell 55:601–610CrossRefGoogle Scholar

Copyright information

© Birkhäuser Boston 1994

Authors and Affiliations

  • Heinz Reichmann
  • Bernd Janetzky
  • Peter Riederer

There are no affiliations available

Personalised recommendations