Senataxin (SETX) is an RNA:DNA helicase mutated in Axonal neuropathy type 2 (AOA2) and Amyotrophic Lateral Sclerosis type 4 (ALS4) patients36,37, with functions at the interface between transcription and genome maintenance pathways35,38–43. Here, SETX is linked to amyotrophic lateral sclerosis type 4.