TDP43 autoregulation gives rise to dominant negative isoforms that are tightly controlled by transcriptional and post-translational mechanisms
TDP43 mislocalization and dysfunction are characteristic of ALS, FTLD, and related neurodegenerative diseases. Dykstra et al. uncover pre- and post-translational mechanisms responsible for regulating cytoplasmic, aggregation-prone shortened (s)TDP43 isoforms. They also show that sTDP43 inhibits full-length TDP43, providing key insights into native pathways for maintaining TDP43 homeostasis.
