Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in

SETX ALS4 mice thus recapitulated ALS disease phenotypes in association with TDP-43 mislocalization and provided insight into the basis for TDP-43 histopathology, linking SETX dysfunction to common pathways of ALS motor neuron degeneration.

You can read the full paper here: https://doi.org/10.1007/s00401-018-1852-9