Brain proteasomal function in sporadic Parkinson's disease and related disorders

Ann Neurol. 2002 Jun;51(6):779-82. doi: 10.1002/ana.10207.

Abstract

Because genetic defects relating to the ubiquitin-proteasome system were reported in familial parkinsonism, we evaluated proteasomal function in autopsied brains with sporadic Parkinson's disease. We found that proteasome peptidase activities in a fraction specific to the proteasome were preserved in five brain areas (including the striatum) of Parkinson's disease where neuronal loss is not observed. Striatal protein levels of two proteasome subunits were normal in Parkinson's disease but reduced mildly in disease controls (multiple system atrophy). Our brain data suggest that a systemic, global disturbance in the catalytic activity and degradation ability of the proteasome itself is unlikely to explain the cause of Parkinson's disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Brain / enzymology*
  • Brain / physiology
  • Cysteine Endopeptidases / genetics*
  • Cysteine Endopeptidases / metabolism*
  • Humans
  • Multienzyme Complexes / genetics*
  • Multienzyme Complexes / metabolism*
  • Multiple System Atrophy / enzymology*
  • Multiple System Atrophy / genetics
  • Parkinson Disease / enzymology*
  • Parkinson Disease / genetics
  • Peptide Hydrolases / metabolism
  • Proteasome Endopeptidase Complex
  • Supranuclear Palsy, Progressive / enzymology*
  • Supranuclear Palsy, Progressive / genetics

Substances

  • Multienzyme Complexes
  • Peptide Hydrolases
  • Cysteine Endopeptidases
  • Proteasome Endopeptidase Complex