人体SOD1突变体在酵母细胞模型中的毒性功能的发现和研究.pdfVIP

foundtocausePmal-GFP mislocalization．FortheG4lDandG41Smutantcells． Pmal一GFPwas foundtobelocalizedeither in membraneormislocalizedas correctly inthe or 11 l 3Tmutantcells．InadditiontoPmal．GFP A4V,G85R．G93C and1113Tmutant mislocalization，A4V cellswerefoundtobe significantlyenlarged insize． SimilartOthe I G85R Cu，ZnSOD cells yeast mutant，yeast expressing humanI113T I mutant withdarkbrowncolor Cu，ZnSODdisplayedcrinklyphenotype andcellswerefilamentousin drastic indicate shape．The morphologicalchanges thatthe l11 human3Tand G85Rmutants thecriticalcell yeast perturbed cycle currentwork process．The showsforthefirsttimethatthehuman fALS．causing l113Tmutationin indeed a Cu，ZnSODl toxicfunctionandinduceddramatic gained andcelldeath．The thetoxic morphologicalchanges results，therefore，proved forfALSandfurtherdemonstratethat isan gain—of-functionhypothesis yeast modelfor fALS． important studying Keywords：Cu，ZnSOD I，fALS，yeast，Pmal，morphology IV 目录