Mitochondrial function, GSH and iron in neurodegeneration and Lewy body diseases

J Neurol Sci. 1998 Jun 11;158(1):24-9. doi: 10.1016/s0022-510x(98)00095-1.

Abstract

The cause of neuronal loss in patients with idiopathic Parkinson's disease is unknown. Oxidative stress and complex I deficiency have both been identified in the substantia nigra in Parkinson's disease but their place in the sequence of events resulting in dopaminergic cell death is uncertain. We have analysed respiratory chain activity, iron and reduced glutathione concentrations in Parkinson's disease substantia innominata and in the cingulate cortex of patients with Parkinson's disease, Alzheimer's disease and dementia with Lewy bodies to investigate their association with neuronal death and Lewy body formation. No abnormalities of mitochondrial function, iron or reduced glutathione levels were identified in Parkinson's disease substantia innominata or cingulate cortex. Mitochondrial function also appeared to be unchanged in cingulate cortex from patients with Alzheimer's disease and from patients with dementia with Lewy bodies, however, iron concentrations were mildly increased in both, and reduced glutathione decreased only in Alzheimer's disease. These results confirm the anatomic specificity of the complex I deficiency and decreased levels of reduced glutathione within the Parkinson's disease brain and suggest that these parameters are not associated with cholinergic cell loss in Parkinson's disease nor with Lewy body formation in this or other diseases. We propose that our data support a 'two-hit' hypothesis for the cause of neuronal death in Parkinson's disease.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Alzheimer Disease / etiology
  • Alzheimer Disease / metabolism*
  • Alzheimer Disease / pathology
  • Dementia / etiology
  • Dementia / metabolism
  • Dementia / pathology
  • Dopamine / metabolism
  • Energy Metabolism
  • Glutathione / analysis
  • Glutathione / deficiency*
  • Gyrus Cinguli / chemistry*
  • Gyrus Cinguli / pathology
  • Humans
  • Iron / analysis*
  • Lewy Bodies / chemistry*
  • Middle Aged
  • Mitochondria / enzymology
  • Mitochondria / physiology*
  • Models, Neurological
  • NAD(P)H Dehydrogenase (Quinone) / analysis
  • NAD(P)H Dehydrogenase (Quinone) / deficiency*
  • Nerve Degeneration / metabolism*
  • Nerve Tissue Proteins / analysis
  • Nerve Tissue Proteins / deficiency*
  • Neurons / metabolism
  • Neurons / pathology
  • Organ Specificity
  • Oxidative Stress
  • Parkinson Disease / etiology
  • Parkinson Disease / metabolism*
  • Parkinson Disease / pathology
  • Substantia Innominata / chemistry*
  • Substantia Innominata / pathology
  • Substantia Nigra / chemistry*
  • Substantia Nigra / pathology

Substances

  • Nerve Tissue Proteins
  • Iron
  • NAD(P)H Dehydrogenase (Quinone)
  • Glutathione
  • Dopamine