Nonetheless, humanization of Stmn2 in ALS model mice more closely resembling human TDP-43 neuropathology may represent an excellent approach to quantify the contribution of TDP-43 induced reduction of Stmn2 protein to the degenerative phenotype, and may simultaneously provide an ideal model to determine potential benefits of restoring Stmn2 expression using ASOs. Here, TARDBP is linked to amyotrophic lateral sclerosis.