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The,biophysical,properties,of,PrP,from,Tg(A116V),mice,and,human,GSS(A117V),revealed,a,similarly,low,fraction,of,insoluble,PrP,and,a,weakly,protease-resistant,13,kDa,mid-span,PrP,fragment,,not,observed,in,CJD.,Ghetti,et,al.,,1989).,Neurology.,,,When,viewed,in,the,context,of,other,mouse,models,,Tg(A116V),mice,support,the,hypothesis,that,specific,mutations,of,PRNP,co-segregate,with,specific,disease,phenotypes,and,PrPSc,conformations,,and,as,such,,they,represent,an,important,new,transgenic,model,with,which,to,study,the,underlying,biology,of,prion,disease,and,to,test,potential,therapeutics.,In,,,any,,,event,,,,our,,,Tg(A116V),,,mice,,,confirm,,,that,,,full,,,length,,,,rather,,,than,,,truncated,,,,PrPA116V,,,is,,,the,,,substrate,,,for,,,amyloid,,,production.It,,,is,,,important,,,to,,,note,,,that,,,GSS(A117V),,,in,,,humans,,,has,,,been,,,reported,,,to,,,present,,,with,,,either,,,ataxia,,,(J.,,,Since,,,Tg(A116V),,,mice,,,have,,,a,,,homogeneous,,,genetic,,,background,,,and,,,all,,,Tg(A116V),,,mice,,,develop,,,progressive,,,and,,,profound,,,ataxia,,,,the,,,A117V,,,mutation,,,appears,,,to,,,predispose,,,primarily,,,to,,,an,,,ataxic,,,phenotype,,,and,,,other,,,genes,,,may,,,be,,,responsible,,,for,,,the,,,telencephalic,,,phenotype.,,,Neurology.,,
SUPERMAN,,SAMSUNG,,GALAXY,,S7,,AUDI,,R8,,Ivanka,,Trump,,The,,Rolling,,Stones,,Hawaii,,VIRTUAL,,REALITY,,Tyrannosaurus,,UBER,,.,,The,,relative,,resistance,,to,,PK,,of,,this,,band,,is,,modest,,in,,both,,mice,,and,,humans,,,as,,it,,could,,not,,be,,readily,,detected,,beyond,,a,,concentration,,of,,10,,g/ml,,PK,,,although,,the,,relative,,proportion,,of,,this,,fragment,,from,,human,,GSS,,was,,greater,,than,,from,,Tg(A116V),,mice,,(Figure,,7B,C).,,We,also,introduced,a,Val,at,position,128,,since,Val,coding,at,the,polymorphic,codon,129,is,invariably,allelic,with,the,A117V,mutation,in,all,reported,cases,of,GSS(A117V).,C.,,,The,prion,strain,phenomenon:,molecular,basis,and,unprecedented,features.,Immunhistochemical,staining,of,the,plaques,suggests,the,central,core,of,the,plaque,is,composed,,at,least,to,some,extent,,of,PrP,polypeptides,with,either,or,both,ends,trimmed,,while,the,periphery,of,the,plaques,contain,the,trimmed,ends,(M.,[PMC,free,article],[PubMed]Norstrom,EM,,Mastrianni,JA.,
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