Dr. Rothstein works in ALS research since a long time (~20 years?). But what is presented here is not very impressive. Working with iPSC-derived is something that could be done at little cost now, and (fortunately) many teams are working on ways to mitigate TDP-43 aggregates.
And it only pushes the goal post further away: What makes CHMP7 aggregate in the nucleus?
I guess this work has two objectives:
1. Participating in the run to patent TDP-43 therapies.
2. Showing to funding parties that the lab is doing something with their money.