Proteasomal dysfunction in sporadic Parkinson's disease.
The cause and mechanism of neuronal death in sporadic Parkinson's disease (PD) continue to elude investigators. Recently, alterations in proteasomal function have been detected in the brain of patients with the illness. The biochemical basis of the defect and its relevance to the disease process are now being studied. The available results suggest that proteasomal dysfunction could underlie protein accumulation, Lewy body formation, and neuron death in PD. The cause of proteasomal dysfunction is unknown at present, but this could relate to gene mutations, oxidative damage, ATP depletion, or the actions of environmental toxins. It remains to be established if proteasomal dysfunction plays a primary or a secondary role in the initiation or progression of the neurodegenerative process in PD.
McNaught
Kevin St P
KS
Department of Neurology, Mount Sinai School of Medicine, New York, NY 10029, USA. kevin.mcnaught@mssm.edu
Jackson
Tehone
T
JnoBaptiste
Ruth
R
Kapustin
Alexander
A
Olanow
C Warren
CW
D001921:Brain / Q000503:physiopathology*; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D008959:Models, Neurological; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D009474:Neurons / Q000378:metabolism*; D010300:Parkinson Disease / Q000209:etiology* / Q000503:physiopathology*; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism*
D009419:Nerve Tissue Proteins; D046988:Proteasome Endopeptidase Complex