Conversely, the depletion of ATXN2 by knock-out or by injection of antisense-oligonucleotides has a massive neuroprotective effect in yeast/fly/mouse models of ALS and FTLD, as well as in SCA2 and SCA1 fly models [24,27,28,29]. Here, ATXN2 is linked to amyotrophic lateral sclerosis.