Quantitative immunoprecipitation demonstrated that SOD1-knockdown HEK293 cultures show a highly significant 15–25 fold drop in misfolded HuWtSOD1 compared to non-knockdown HEK293 cells when incubated with conditioned media originating from mutant FUS, as well as wild-type and mutant TDP-43, transfected cells ( Fig. 2e,f ).
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TDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashion.
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