FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration 英文参考文献.docVIP

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FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration 英文参考文献.doc

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FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration 英文参考文献

FUSTransgenicRatsDevelopthePhenotypesof AmyotrophicLateralSclerosisandFrontotemporalLobar Degeneration CaoHuang1.,HongxiaZhou1.*, JianbinTong1,HanChen2,Yong-JianLiu3,DianWang1,XiaotaoWei1, Xu-GangXia1* 1DepartmentofPathology,Anatomy,andCellBiology,ThomasJeffersonUniversity,Philadelphia,Pennsylvania,UnitedStatesofAmerica,2CenterforBiotechnology, University of Nebraska–Lincoln, Lincoln, Nebraska, United States of America, 3Department of Neurobiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania,UnitedStatesofAmerica Abstract FusedinSarcoma(FUS)proteinopathyisafeatureoffrontotemporallobardementia(FTLD),andmutationofthefusgene segregateswithFTLDandamyotrophiclateralsclerosis(ALS).Tostudytheconsequencesofmutationinthefusgene,we created transgenic rats expressing the human fus gene with or without mutation. Overexpression of a mutant (R521C substitution), but not normal, human FUS induced progressive paralysis resembling ALS. Mutant FUS transgenic rats developedprogressiveparalysissecondarytodegenerationofmotoraxonsanddisplayedasubstantiallossofneuronsin the cortex and hippocampus. This neuronal loss was accompanied by ubiquitin aggregation and glial reaction. While transgenicratsthatoverexpressedthewild-typehumanFUSwereasymptomaticatyoungages,theyshowedadeficitin spatiallearningandmemoryandasignificantlossofcorticalandhippocampalneuronsatadvancedages.Theseresults suggestthatmutantFUSismoretoxictoneuronsthannormalFUSandthatincreasedexpressionofnormalFUSissufficient toinduceneurondeath.OurFUStransgenicratsreproducedsomephenotypesofALSandFTLDandwillprovideauseful modelformechanisticstudiesofFUS–relateddiseases. Citation: Huang C, Zhou H, Tong J, Chen H, Liu Y-J, et al. (2011) FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and FrontotemporalLobarDegeneration.PLoSGenet7(3):e1002011.doi:10.1371/journal.pgen.1002011 Editor:GregoryCox,TheJacksonLaboratory,UnitedStatesofAmerica ReceivedOctober5,2010;AcceptedJanuary3,2011;Pu