Figure 4.

Experimental paradigms demonstrating the prion-like properties of SOD1 and TDP-43 in mice. This schema is a compilation of several published results obtained by the administration of misfolded SOD1 and TDP-43 aggregates in different mouse models. The nature of protein extracts (crude homogenates, purified proteins, recombinant proteins), the route of delivery and the genetic background of injected mice are indicated. For the observed physiopathological outcome, three criteria are mentioned: the levels of protein inclusions/aggregates in the CNS, the motor symptoms evaluated by behaviour tests and the lifespan of injected mice.