Brain proteasomal function in sporadic Parkinson's disease and related disorders.
Identifieur interne : 003421 ( Main/Merge ); précédent : 003420; suivant : 003422Brain proteasomal function in sporadic Parkinson's disease and related disorders.
Auteurs : Yoshiaki Furukawa [Canada] ; Sophie Vigouroux ; Henry Wong ; Mark Guttman ; Ali H. Rajput ; Lee Ang ; Marièle Briand ; Stephen J. Kish ; Yves BriandSource :
- Annals of neurology [ 0364-5134 ] ; 2002.
English descriptors
- KwdEn :
- 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).
- MESH :
- chemical , genetics : Cysteine Endopeptidases, Multienzyme Complexes.
- enzymology : Brain, Multiple System Atrophy, Parkinson Disease, Supranuclear Palsy, Progressive.
- genetics : Multiple System Atrophy, Parkinson Disease, Supranuclear Palsy, Progressive.
- chemical , metabolism : Cysteine Endopeptidases, Multienzyme Complexes, Peptide Hydrolases.
- physiology : Brain.
- Aged, Humans, Proteasome Endopeptidase Complex.
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.
DOI: 10.1002/ana.10207
PubMed: 12112087
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pubmed:12112087Le document en format XML
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<author><name sortKey="Kish, Stephen J" sort="Kish, Stephen J" uniqKey="Kish S" first="Stephen J" last="Kish">Stephen J. Kish</name>
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<front><div type="abstract" xml:lang="en">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.</div>
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