Neurobiology of aging · 2008
Cell-produced alpha-synuclein oligomers are targeted to, and impair, the 26S proteasome.
СтатьяПоддержка NIH: внешняя программаПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Emmanouilidou Evangelia, Stefanis Leonidas, Vekrellis Kostas
Аннотация
Proteasomal dysfunction may play a role in neurodegenerative conditions and protein aggregation. Overexpression in neuronal cells of alpha-synuclein, a molecule linked to Parkinson's Disease, may lead to proteasomal dysfunction. Using PC12 cells stably expressing wild-type or mutant alpha-synuclein and gel filtration, we demonstrate that soluble, intermediate size oligomers of alpha-synuclein co-elute with the 26S proteasome. These soluble oligomers associate with the 26S proteasome and are significantly increased following treatment with proteasomal, but not lysosomal, inhibitors, indicating specific degradation of these particular species by the 26S proteasome. Importantly, expression of alpha-synuclein resulted in a significant inhibition of all proteasomal activities without affecting the levels or assembly of the 26S proteasome. Pharmacological dissociation of alpha-synuclein oligomers restored proteasomal function and reduced polyubiquitinated protein load in intact cells. Our findings suggest a model where only a subset of specific soluble cell-derived alpha-synuclein oligomers is targeted to the 26S proteasome for degradation, and simultaneously inhibit its function, likely by impeding access of other proteasomal substrates.
Предметные рубрики MeSH
AlanineAmmonium ChlorideAnimalsBenzoquinonesCerebral CortexChromatography, GelColoring AgentsCongo RedEnzyme InhibitorsGene Expression Regulation, EnzymologicGreen Fluorescent ProteinsHumansImmunoprecipitationLactams, MacrocyclicMiceMice, Inbred C57BLMice, TransgenicMutationPC12 CellsProteasome Endopeptidase ComplexProtein FoldingRatsTransfectionTyrosinealpha-Synuclein