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Expression of human FUS/TLS in yeast leads to protein aggregation and cytotoxicity,recapitulating key features of FUS proteinopathy

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Protein & Cell 2011年第2期2卷 141-149页

作者： Kazuo Fushimi Charles Long neha jayaram Xiaoping Chen Liming Li Jane Y.Wu Department of Neurology Center for Genetic MedicineLurie Comprehensive Cancer CenterNorthwestern University Feinberg School of Medicine303 E.SuperiorChicagoIL 60611USA Department of Molecular Pharmacology and Biological Chemistry Northwestern University Feinberg School of Medicine303 E.SuperiorChicagoIL 60611USA

Mutations in the fused in sarcoma/translocated in liposarcoma(FUS/TLS)gene have been associated with amyotrophic lateral sclerosis(ALS).FUS-positive neuropathology is reported in a range of neurodegenerative diseases,including ALS and fronto-temporal lobar degeneration with ubiquitin-positive pathology(FTLDU).To examine protein aggregation and cytotoxicity,we expressed human FUS protein in *** of either wild type or ALS-associated R524S or P525L mutant FUS in yeast cells led to formation of aggregates and cytotoxicity,with the two ALS mutants showing increased ***,yeast cells expressing human FUS protein recapitulate key features of FUSpositive neurodegenerative ***,a significant fraction of FUS expressing yeast cells stained by propidium iodide were without detectable protein aggregates,suggesting that membrane impairment and cellular damage caused by FUS expression may occur before protein aggregates become microscopically detectable and that aggregate formation might protect cells from FUS-mediated *** N-terminus of FUS,containing the QGSY and G rich regions,is sufficient for the formation of aggregates but not *** C-terminal domain,which contains a cluster of mutations,did not show aggregation or *** to TDP-43 when expressed in yeast,FUS protein has the intrinsic property of forming aggregates in the absence of other human *** the other hand,the aggregates formed by FUS are thioflavin T-positive and resistant to 0.5%sarkosyl,unlike TDP-43 when expressed in yeast ***,TDP-43 and FUS display distinct domain requirements in aggregate formation and cytotoxicity.

关键词： FUS/TLS protein aggregation cytotoxicity

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