Ubiquitin-specific peptidase-19 links TDP-43 aggregation to ER stress

Proceedings of the National Academy of Sciences, Volume 123, Number 11, March 2026.
SignificanceThe pathological hallmark of FTLD-TDP and ALS is cytoplasmic deposition of TDP-43, a process dependent on the ubiquitin-proteasome system. Furthermore, ER stress promotes cytoplasmic aggregation of TDP-43, which in turn reciprocally induces the ER…